A system of practical medicine. By American authors. Vol. 2

by Unknown

HEREDITARY SYPHILIS.

BY J. WILLIAM WHITE, M.D.

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About a half century ago Mr. Abraham Colles, who had just resigned the professorship of the Theory and Practice of Surgery in the Royal College of Surgeons in Ireland, the duties of which, in the opinion of the college, he had discharged for thirty-two years in an "exemplary and efficient manner," wrote the following introductory paragraph to his remarkable chapter on "Syphilis in Infants:" "Perhaps there is not in the entire range of surgical diseases any one the contemplation of which is more calculated to arrest our attention or to excite our interest than syphilis infantum."

Although it was not then, and is not at the present day, strictly relegated to the domain of surgery, hereditary syphilis, like its parent disease, was generally treated of by the practitioner of that branch of medicine. And yet in the great majority of instances the management of such cases, especially as regards their family relations, the relations of husband and wife, the management of the latter during pregnancy, the delivery and subsequent care of the child, the necessary attention to the safety of other members of the family—in fact, all of the most weighty responsibility—falls upon the ordinary medical attendant. It is therefore in every way proper that the condition should receive some notice in a system of general medicine.

A proper presentation of the subject of hereditary syphilis involves a consideration of the vexed question as to the mode by which the disease is conveyed from parent to offspring. That it may be so transmitted has been generally believed since the doctrine was first announced by Torella at the end of the fifteenth century; and the facts in its support are so numerous and convincing that, in spite of a few distinguished opponents—among whom John Hunter was the most conspicuous¹—it has been unhesitatingly accepted by the profession down to the present day. As regards the manner of transmission, however, controversy has been and still is rife. Opposing theories have been constructed and ardently supported, differing radically as to essential points, often resting upon exceptional or anomalous, and still oftener upon imperfectly observed, cases.²

¹ Works of John Hunter, vol. ii. p. 383.

² Parrot, in a clinical lecture on syphilitic abortion (Le Progrès Médicale, Nov. 3, 1877, p. 798), says: "The infection of children was known, but its true origin was not suspected. The belief of Gaspard Torella (1498) and Matthioli (1536) that it came from the nurses through the milk was generally accepted."

According to Diday, Paracelsus (1529) was the first to plainly state the heredity of syphilis: "Fit morbus hereditarius et transit a patre ad filium."

Others attribute the original announcement to Augier Ferrier (1553), and it seems certain that he was first to specify the three modes of infection of the product of conception: "La semence du père, celle de la mère, et la contamination de la mère durant la grossesse."

Fallopius in a posthumous treatise on the Mal Français (1566) adds the authority of his name to this view: "Præterea videbitis puerulos nascentes ex foemina infecta, ut ferant peccata parentum, qui vedentur semi cocti."

Ambroise Paré also acquiesced in the theory, saying, "Souvent on voir sortir les petits enfants hors le ventré de leur mère, ayant ceste maladie, et tost après avoir plusieurs pustules sur leur corps; lesquels étant ainsi infectés, baillent la vérolle à autant de nourrices qui les allaictent."

Subsequently, Mauriceau, Boerhaave, and Astruc sustained the same view, which, with the single exception of Hunter, had no prominent antagonist.

It was not, however, until the eighteenth century that it was described with any attempt at detail or exactness by Rosenstein, and his essay is loaded with errors. It was in the foundling hospitals of Paris at the end of the last century, in the wards of Salpétrière and Bicêtre, and in the hospitals of Vaugirard and in the Capucin convents of the Rue Saint Jacques, where pregnant women and nurses attacked with syphilis were admitted, that methodical and trustworthy observations were made (1780-1810) by Colombier, Despenières, Doublet, Mahon, Cullerier, and Bertin. Since then the history of the disease has been the history of syphilis itself.

A full consideration of these, or even a recapitulation of the respective arguments pro and con, would far exceed the limits allotted to the present article, and we will confine ourselves simply to stating the questions which most nearly concern the practical physician, and the conclusions which the accumulated observation and experience of the profession seem to justify. The points bearing upon the general subject of hereditary syphilis which exercise an important influence upon advice or opinions of the utmost gravity as regards the happiness and well-being of the individual or family may be enumerated as follows:

1. Is syphilis in all its stages transmissible (a) to the wife or husband, (b) to the offspring? Or, in other words, is it ever proper to consent to the marriage of a person who has had syphilis? If so, under what circumstances?

2. By what means or through what channels can the disease of the parents reach the child?

3. What are the pathology and symptoms of hereditary syphilis?

4. What is the treatment—(a) prophylactic, applied to the parents, and (b) curative? We may now take these up seriatim.

No more important questions can be submitted to a medical man than those pertaining to the marriage of syphilitics. Involving as it does the welfare of many individuals, modifying or fixing the conditions or circumstances of one or more lives, his opinion should be exceptionally definite and well grounded. The responsibility of advising or consenting to the marriage of a person who has once had syphilis is undoubtedly great; the responsibility of prohibiting it is, however, no less so. Matrimony is the natural condition for the majority of people. Enforced celibacy, especially in males, brings with it not infrequently a long train of attendant evils, moral and physical. It will not do to assume that professional duty is properly discharged by telling all patients to be on the safe side and to remain single for fear of inoculating wife or offspring, unless it can be clearly shown that there is a definite and unavoidable risk in every case, which continues throughout life.³

³ "The surgeon who, on account of past syphilis, forbids marriage to an otherwise eligible man must remember that he forbids it at the same time to some woman, who, possibly, if well informed as to her risks, would willingly encounter them.... Respecting a malady so common as syphilis, while it is often our duty to warn, it is also not unfrequently our duty to encourage" (Mr. Hutchinson, preface to the English translation of Fournier's Syphilis and Marriage, p. vii.)

There are two distinct methods of arriving at an answer to the question under discussion: first, by considering the probabilities in regard to the essential nature of syphilis; and, second, by carefully weighing the clinical evidence in the matter. It seems evident that belief in any particular theory of syphilis assigning it to this or that class of disease must have an important influence in determining the opinion which is held as to its curability, or at least as to its indefinite transmissibility.

If the late symptoms, the so-called tertiary outbreaks of the disease, are held to be evidences of the presence in the system of the specific virus, which has simply remained for a long period, perhaps for many years, latent or quiescent, and which is thus again manifesting its power; if syphilis is believed, accordingly, to be a practically unlimited disease, conforming to no known law as regards its duration, corresponding to no other infectious or contagious malady in having a period of termination—more or less delayed perhaps, or more or less indefinite, but still invariably present—at which time either the particular poison or the susceptibility of the system to its influence has become exhausted;—if tertiary syphilis, in other words, is regarded as simply a continuance or recurrence of the disease, differing in no essential respect, except as to the particular tissues involved, from the same disease in its early stages, it is difficult to see how marriage can ever be conscientiously recommended to a person who has once contracted it.

It is unfortunately true that in no given instance is it safe to assure a patient of further complete immunity from the disease. In any case, however mild in its course or under whatever treatment, there is always an element of doubt as to the development of subsequent symptoms. The probability of their appearance may be reduced to a minimum, the character of the case and the thoroughness of the treatment may both seem to give assurance that a cure has been completed, and yet both patient and physician may be mortified and annoyed by an outbreak of tertiary lesions. This, I think, would be admitted by every one of large experience with the disease, and indeed furnishes the chief argument to those who deny or are sceptical as to its curability. If, then, it were impossible to predict with any sort of certainty that the contagious and highly transmissible stage of syphilis would terminate during the life of the individual, it would manifestly be unwise to permit marriage, with its risk of inoculating the innocent partner and the consequent double risk to the offspring.

If, however, syphilis is, as taught by Mr. Hutchinson,⁴ one of the exanthemata, having, like them, a period of outbreak, a period of efflorescence or eruption, and a period of subsidence, and followed, like them, by certain non-contagious sequelæ, which we call the tertiary symptoms, but which are merely relapses or degenerations of parts affected during the secondary stage, it becomes evident that the risk of transmission to wife or husband or children after the lapse of a certain interval becomes greatly reduced or almost nil. No one thinks of forbidding marriage on account of a previous attack of small-pox or scarlet fever or typhoid fever, even although there are unpleasant consequences which sometimes follow these diseases.

⁴ The London Lancet, Feb. 5, 1876; Reynolds's System of Medicine, Am. ed., p. 423.

Or if another and still more plausible theory of syphilis is adopted, and we look on the tertiary period as one of contraction or obliteration of lymphatics due to long-continued irritation by the new cell-growths of the secondary stage, which from the very onset affect those vessels, our views will be materially modified. During the primary period, then, when the new cells, which either constitute the poison of syphilis or are its carriers, are accumulating at the site of original inoculation, constituting the induration of the chancre, or are slowly finding their way into the general system through the lymphatic vessels, proliferating in their walls and thickening and hardening them, or during the secondary period, when they are rapidly multiplying in all the tissues of the body, the risk of inoculation or transmission would be manifestly great. When, however, by destructive metamorphosis and degeneration, either with or without the aid of drugs, they have been eliminated from the body, the contagious element disappears with them; and although here and there throughout the body some important lymphatic trunk may have undergone irreparable injury, and may have been contracted or obliterated, permitting of the accumulation of waste products until the node or gumma or tubercle which we call a tertiary symptom makes its appearance, yet the disease has lost much of its terror, and has become dangerous only to the patient himself.⁵

⁵ Clinical Lectures on the Physiological Pathology and Treatment of Syphilis, by F. N. Otis, M.D.; Syphilis, by V. Cornil, Am. ed., 1882, pp. 17-27.

These theories are only alluded to by way of elucidation of the statement that belief in one or the other of them has an important bearing on the relation of syphilis to marriage, and because, whichever is thought to be the most plausible, they equally lessen or altogether do away with a certain proportion of the danger formerly thought to surround the marriage of a syphilitic even after a most protracted interval. It is not necessary to accept either the one or the other implicitly. The essential point is the recognition of the fact that modern syphilographers, as a rule, regard the tertiary or late symptoms as indicative of damage done during the active period—as relapses or sequelæ, and not as fresh outbreaks, of a highly contagious and transmissible disease. Their time of appearance, their entire want of symmetry, their non-contagiousness, their non-inoculability, all favor this view, and we may now see what evidence corroborative of it may be obtained from clinical facts.

It will be necessary, in the first place, to admit that there seems to be but little doubt in the minds of most syphilographers that in rare instances syphilitic children have been born to parents who had long passed the limits of the secondary period. At least the great majority of writers upon this subject speak confidently of the exceptional occurrence of such cases, and assert that syphilis may be transmitted during any of its stages.⁶ If, however, we come to look for positive evidence in this respect, we will find very little that is entirely satisfactory. Cases are reported, to be sure, in which eight, ten, twelve, or even fifteen or twenty, years after the primary sore, syphilitic patients have become the parents of children who showed unmistakable indications of the disease. When we examine the history of the cases, we find usually that many important points are omitted without which it is impossible to be certain of its true character. Were both parents originally infected? If not, has a recent case of syphilis occurred in the one who at first escaped? If they were both diseased originally, has either been subsequently re-infected?—a much more frequent accident than has been commonly supposed.⁷ On applying these tests to the cases in question it will be found that few if any of them are thoroughly convincing. Kassowitz's observations,⁸ made, it must be remembered, upon persons with whom no mercurial treatment had been employed, seemed to show that the average limit of transmissibility was about ten years, after which time healthy children began to be born. His observations were, however, incomplete in many respects, and, like all such investigations, are of course open to the suspicion of intentional deception on the part of the patients.⁹ Even these cases, however, show unequivocally, as do all which have ever been recorded, the steady diminution of the transmissive power under the influence of time alone.

⁶ Belhomme et Martin, Traité de la Syphilis, p. 413.

⁷ Cornil, op. cit., p. 20.

⁸ Die Vererbung der Syphilis, Wien, 1876.

⁹ See also Hutchinson, British and Foreign Med.-Chir. Rev., Oct., 1877.

I have said that the majority of writers seem to have no doubt of the long continuance of this transmissive power in rare cases. There are, however, a few notable exceptions. Fournier, whose immense experience and acuteness of observation entitle his opinion to the utmost consideration, says that in cases of paternal heredity the duration of the force of transmission never exceeds at the maximum three or four years.¹⁰ In no case of the many hundreds he has observed has he known a syphilitic father to infect a child—the mother being healthy—at a later period than the one mentioned. And he is equally positive that the gradual diminution and final extinction of the syphilitic reaction of the parents upon the children is a veritable pathological law, "absolutely demonstrated."¹¹

¹⁰ Syphilis and Marriage, Am. ed., p. 87.

¹¹ Op. cit., p. 88. Of course when both parents are diseased a somewhat longer period of activity is to be expected for the poison.

M. Mireur, a careful and accurate observer, records¹² a striking instance in which in the history of a couple, both syphilitic and untreated, eight pregnancies occurred. The first resulted in abortion at fifth month; the second, in abortion at seventh month; the third, in a stillbirth; the fourth, a syphilitic child dying in one month; the fifth, in a syphilitic child dying in forty-five days; the sixth, seventh, and eighth, in living, healthy children. To me the most interesting fact in the whole relation is that during a portion of the time, and immediately after the last three pregnancies, which resulted in the birth of healthy children, both husband and wife manifested grave tertiary syphilitic symptoms—gummata, tubercles, ulcers, etc. This is direct evidence of the strongest kind in favor of the view that syphilis ceases to be transmissible by heredity at the end of a certain period, as we know that it ceases to be contagious or inoculable.

¹² Essai sur l'Hérédité de la Syphilis, Thèse de Paris, 1867, p. 91.

M. Ricord long ago,¹³ and even before him Astruc and Doublet,¹⁴ had promulgated the same theory, Ricord asserting that in the tertiary stages the only effect of the disease upon the children was so to modify their organization and temperament as to expose them to developments of a scrofulous character—a view of the relation between syphilis and struma which has been so ably supported in our own day by Professor Gross.

¹³ Traité pratique des Maladies vénériennes Paris, 1838, p. 644.

¹⁴ Legendre, Nouvelles Recherches sur les Syphilides, 1841 (quoted by Belhomme et Martin).

M. Bazin also¹⁵ denies absolutely that tertiary syphilis is any more transmissible than it is communicable in other ways, although he fails to give his reasons for this belief.

¹⁵ Leçons sur les Syphilides, 1859, p. 35.

Hill and Cooper state¹⁶ that the transmissive power continues as long as the secondary eruptions are present, but usually ceases when the tertiary stage is reached.

¹⁶ Syphilis and Local Contagious Disorders, London, 1881, p. 62.

Van Buren and Keyes¹⁷ believe that fathers with tertiary syphilis certainly, as a rule, procreate non-syphilitic children; and in speaking of the fact that when the mother has syphilis the child is generally infected, they except the later tertiary stages.

¹⁷ Genito-urinary Disease and Syphilis, 1874, p. 521.

Bumstead¹⁸ and Taylor say that without mercurial treatment the danger of transmitting the disease to offspring usually persists up to the fourth year of syphilitic contagion.

¹⁸ Venereal Diseases, 1879, p. 739.

Mr. Lane says:¹⁹ "It is certainly the rule that when the parents have fully reached the tertiary stage the children born to them are free from all signs of syphilis."

¹⁹ Lectures on Syphilis, London, 1881, p. 65.

Mr. Hutchinson says:²⁰ "It is almost an acknowledged law that parents in the late tertiary stages do not transmit taint."

²⁰ The Med. Press and Circular, Aug. 2, 1882, p. 85.

It will be seen from the foregoing extracts, which might be greatly multiplied,²¹ that there is a strong tendency on the part of many authors to limit more or less strictly the period of transmissibility of syphilis even when the disease is allowed to progress without treatment. As to the facts that it becomes milder with time, both in parents and offspring, that it ceases to be conveyed from husband to wife or vice versâ, that with each succeeding year after the termination of the secondary period the chances of escape of the product of conception increase in a rapidly augmenting ratio,—there is no difference of opinion whatever. Neither is it seriously disputed that the length of time during which the disease remains active, as well as the degree of its activity, may be markedly and beneficially influenced by the administration of mercurial treatment. Under proper medication patients who have rashly or disobediently married in the height of the secondary period have been enabled to escape the danger of transmission either to spouse or offspring—have, in fact, had children born healthy and who never subsequently manifested any symptoms of the disease.

²¹ M. Diday, Traité de la Syphilis des Nouveau-nés, Paris, 1854, p. 183; M. Bertin, Traité de la Maladie vénérienne chez les Nouveau-nés, Paris, 1870, p. 142; M. Bazin, op. cit., p. 164; M. Roger, L'Union Médicale, 1865, t. i. p. 147 (quoted by Fournier).

I may add that my own experience seems to confirm the views which have thus been set forth. I have notes of all my cases occurring in private practice in a large city—some of them, I regret to say, among personal friends or acquaintances, some of them in our own profession—and have repeatedly given permission to men to marry or to resume marital relations after three years or three years and a half of mild mercurial treatment, to which during the last six months or a year had been added iodide of potassium. In many instances healthy children have been born: in none, so far as I know, has the wife or mother been directly infected. There have been a few doubtful cases in which premature deliveries or stillbirths have occurred, but in nearly every such instance there seemed to be other and entirely competent causes for the accident; and in none of them, as I learned from the father or from the obstetrician in attendance, were the children the subjects of unmistakable syphilitic symptoms.

As to the exact time at which it is safe to permit marriage, and as to the proper treatment before and after that event, it is hardly possible in an essay like this to enter into many details. Yet so much is involved in the answer to our first question that it may not be altogether out of place here to indicate briefly the views of the writer as to general methods of treatment. This is the more proper because in every case of suspected syphilis in a new-born child, in every case of threatened or actual abortion or miscarriage in the wife of a man who has at some time in his life had syphilis, these questions will present themselves, and the answers to them will greatly influence not only the diagnosis and prognosis, but even the treatment, of such cases.

1. In the first place, then, the diagnosis of syphilis should have been assured. No venereal sore can with certainty be pronounced to be syphilitic before the occurrence of general constitutional symptoms, either the early cutaneous eruptions or at least the general glandular involvement. Treatment begun prior to these developments leaves the whole case open to the suspicion of mistaken diagnosis.²²

²² "It is unsafe to predict confidently that any venereal ulcer, even a soft sore attended with suppurating bubo, will entail no further consequences. There is a strong probability that an indurated sore will prove infecting; and there is a probability, though not nearly so strong, that a soft suppurating sore will not; but exceptions to both these general rules will be met with, and there is really no absolute proof of the infecting nature of any given sore but the fact of infection itself" (Lectures on Syphilis, James S. Lane, London, 1881, p. 23).

2. The drug which should at once be begun when the character of the case is fully recognized is mercury in one of its various forms. It may be given by the mouth, by inunction, by vaporization, by hypodermic injection, according to the preferences of the physician or patient; but, however administered, it should be given in sufficient quantity—i.e. in each case the full physiological dose of that particular patient should be employed. To ascertain this the amount used should have been gradually increased until commencing symptoms of salivation are produced, when it should be diminished about one-half.

3. The quantity which has been thus determined should be given continuously, or stopping only for the management of intercurrent complications, for at least eighteen months. If during this time new syphilitic symptoms make their appearance, the dose should be temporarily raised until they have vanished, when it should be brought down again to the original amount.

4. At the end of eighteen months or two years small doses of iodide of potassium should be added to the mercurial, and this mixed treatment should be persevered in for six months or a year longer, or should be still further prolonged if during that time any evidence of syphilis is seen.

5. At the end, then, of from two to three years, if no symptom has been seen for six months or a year, treatment may be stopped and the patient kept under observation for a year; and if during that time no symptom develops he may consider himself as in all probability cured. Any course of treatment less thorough than this should be set down as insufficient to afford any reasonable presumption of further immunity from the disease.

There is evidence to prove, on the other hand, that this plan of treatment, rigidly carried out, results in the majority of cases in curing the disease, or at any rate in putting the patient in such condition that he may with safety marry and may expect to have healthy children.

To recapitulate: Syphilis after a certain period, not extending much over four years where the disease is allowed to run its own course, and probably much reduced by treatment, ceases to be a contagious disease; and at about the same time or some time after loses, in the majority of cases, its capability of being transmitted.

As there are probably exceptions to the rule that this power of transmission disappears spontaneously within any specified time, it is never safe to trust altogether to the unaided efforts of nature, but a vigorous and sufficient specific treatment must be employed.

Given, however, the lapse of a sufficient time—say from three to four years as a minimum—the history of a proper and continuous plan of treatment, and the absence for a year or more of any specific symptoms whatever, and the risks of marriage are so reduced as probably to warrant a careful physician in permitting it.²³ And conversely, of course—and this constitutes the reason for introducing the foregoing matter into a paper on hereditary syphilis—in any doubtful case where such a history can be elicited, and where all these precautions have been observed, it is improbable that any taint of syphilis has been transmitted.

²³ This refers, of course, to an ordinary case of syphilis. If the symptoms have been unusually grave, if the deeper tissues or the viscera have been seriously involved, if cerebral or spinal complications have occurred, the situation is of course much more grave, and no step should be taken without the most thoughtful deliberation. The work of Fournier already alluded to (Syphilis and Marriage) furnishes an admirable guide under these circumstances.

Mr. Frederick Lowndes, surgeon to the Liverpool Dock Hospital (Lancet, July 8, 1882), says: "Each case must be judged on its own merits. When the constitution is good, and there has been sufficient specific treatment, marriage may be permitted within a much shorter period than M. Fournier suggests, and with safety. Syphilis alone and syphilis combined with scrofula are two very different foes to contend with, and if our patient be of a scrofulous temperament a delay even longer than M. Fournier's may be desirable."

He quotes Dr. Thomas Edward Beatty in an address at the annual meeting of the British Medical Association at Leeds in 1869: "Mercury given to the man when first diseased would, I firmly believe, have prevented this terrible calamity—i.e. the syphilitic infection of the wife; and I would now humbly suggest to all who undertake the treatment of venereal disease that if they have a certainty that their patients will remain celibate all their lives, they may heal up their sores and dispel their eruptions and sore throats in any manner they like, but that they have no right to expose the pure, innocent, high-minded females of society to contamination by marrying men treated without mercury."

Beyond this in positiveness of assertion it is not safe to go. There may be exceptions to these as to most other hygienic or therapeutic rules, but they will surely be of excessive rarity.

Before considering the methods by which syphilis can reach the child from one or the other of its parents, it may be well to mention the modes in which they can infect each other.

The father can derive syphilis from the mother only in the usual way—i.e. by contagion through a breach of surface permitting of the direct absorption of the poison, the development of the disease being attended by the usual phenomena—chancre, lymphatic enlargement, skin eruptions, etc. The woman may—and in the vast majority of cases does—acquire the disease from the husband in a similar manner. But there seems to be good reason for believing that she may also become infected through the medium of the child, who receives its syphilis directly from the father, the mother up to the time of conception having escaped contagion. More than this, it appears to be highly probable²⁴ that no woman ever bears a syphilitic child and remains herself absolutely free from the disease.

²⁴ The strongest argument against the theory that every woman who has had a syphilitic child has herself been infected lies in the existence of cases like the following, several of which have been observed: The wife of a man having active but untreated syphilis gives birth to one or two syphilitic children, she herself developing no symptoms. Later, the husband is placed on mercurial treatment. She then conceives and gives birth to a healthy child. He stops treatment, and she again bears a syphilitic child, which on his resuming his mercurial course is followed by another healthy infant. Such cases certainly indicate that the syphilis of the mother, if any exists, is incapable of transmission to the child, as the effect of the treatment of the father is too direct and unvarying to admit of doubt.

The existence of this form of infection—syphilis by conception—has been vehemently denied by many authors²⁵—by some on the theoretical grounds that as the essential elements or carriers of the syphilitic virus have been shown to be cells or protoplasmic particles, and as, after conception, the embryo is supplied with serum, but not with cells of any kind, it is impossible that syphilis can be conveyed either to or from it;²⁶ by others on reported observations of numerous cases in which mothers who have been delivered of syphilitic children have shown no evidences of the disease.

²⁵ Kassowitz, Von Baerensprung, Bidenkap, and others.

²⁶ Bumstead and Taylor, op. cit., p. 742.

It would appear, however, that, setting aside arguments based on theoretical considerations, the weight of clinical evidence is altogether in favor of the frequent, if not the invariable, contamination of the mother through the medium of the foetus. No physician of large experience in this class of cases can fail to have seen some in which the husband, having had syphilis and having married after an insufficient interval or an imperfect course of treatment, has infected his wife with the disease, although at the time no discoverable symptom is to be found upon his body—no abrasion, sore, mucous patch, no lesion of continuity or suspicious point of any description. An equally careful inspection of the woman will also in such cases be attended by negative results—no initial lesion, no spot of induration, no adenopathy being at all discoverable—and yet she will be found with unmistakable evidences of constitutional syphilis.²⁷ There is a clue to all such cases which will immediately resolve the difficulty. In every instance, providing that no mistake has been made and that both husband and wife are really free—the one from any contagious lesion, the other from any evidence of a present or previous primary sore—it will be found that pregnancy has occurred; that the woman has either been delivered of a syphilitic child or has had an abortion or miscarriage at some time before the outbreak of the symptoms of syphilis. Although I am firmly convinced that this is a statement of facts based upon careful clinical observation, and although this view has received the unqualified endorsement of no less an authority than Fournier,²⁸ it is yet strongly combated by many excellent authorities. They say in reply to the above arguments that the reported cases are open to just criticism, that trifling and unnoticed lesions of the father—chafes or abrasions almost microscopic—suffice to transmit it on his part; while as an explanation of the supposed absence of the primary lesion in the mother they plead the well-known difficulty of discovering it in women under any circumstances.

²⁷ For a typical case see Colles's Works, New Sydenham Society, London, 1881, p. 253. From that date to this hundreds of such cases have been observed, and it would be idle to refer to them.

²⁸ Op. cit., pp. 26-30. He confesses to complete ignorance as to the precise method by which this contagion takes place, whether by poisoning of the fecundated ovule at the moment of conception (the theory of Von Baerensprung) or by exchanges of the utero-placental circulation, and regards the various hypotheses upon these points as without practical value.

Another argument, however, which seems to me to be unanswerable lies in the application to the case in question of the well-known "law of Colles," which from the date of its first enunciation by its distinguished expounder in 1837 down to the present day has been found to be absolutely without exception. I know of no other statement in reference to disease which is at once so sweeping and comprehensive in its bearings and so completely substantiated by clinical experience. It may be given in his own words: "One fact well deserving our attention is this: that a child born of a mother who is without obvious venereal symptoms, and which, without being exposed to any infection subsequent to its birth, shows this disease when a few weeks old,—this child will infect the most healthy nurse, whether she suckle it or merely handle and dress it; and yet this child is never known to infect its own mother, even though she suckle it while it has venereal ulcers of the lips and tongue."²⁹

²⁹ The Works of Abraham Colles, edited by Robert McDonnell, the New Sydenham Society, London, 1881, chap. xiii. p. 287.

As to the absolute and unvarying truth of this law there is no excuse for a shadow of doubt. To quote Mr. Hutchinson: "It has received the assent of every authority who has written on the subject since it was announced. It has attracted attention both at home and abroad, and I am not aware that a single exception to it has been recorded.³⁰ We have all of us seen chancres on the nipples of wet-nurses. They are, indeed, not very infrequent. We have, however, none of us seen such on those of the mothers of infected children. Let us remember that it is very unusual to put a syphilitic infant out to wet-nurse—a thing which no prudent physician would ever permit—and that, probably, for one so nursed a hundred are suckled by their mothers, and we can appreciate the weight which this entire absence of proof that mothers ever suffer bears. It amounts, I think, to all but proof that they are absolutely insusceptible."³¹

³⁰ The cases of Cazenave (1847), Cocchi (1858), Müller (1861), Ranke (1878), Guibout (1879), Scarenzio (1880), and Zingalès (1882), are defective in important particulars, the first two so much so as to render them valueless. Ranke's case is by far the strongest yet recorded, but lacks fulness and rests upon his unsupported testimony. (See Hill and Cooper, op. cit., p. 55, and Nouveau Dict. de Méd. et de Chir., vol. xxxiv. p. 687.)

³¹ Dr. J. N. Hyde suggests (Archives of Dermatology, April, 1878, p. 103) that "the full weight of Colles's law is to be estimated in connection with the question whether the child whose hereditary syphilis is derived from the mother exclusively is capable of infecting its healthy father; and if no instance of this latter can be adduced a higher law becomes defined—viz. that the child whose hereditary syphilis is transmitted by one parent only is incapable of infecting either." He explains this hypothetical immunity, in which he seems to believe, by saying that "it is probably due to the fact that the syphilis-bearing cell-element cannot readily be implanted upon the soil from which it sprang—a fact illustrated by the infecundity of consanguineous marriages and the non-auto-inoculability in general of the primary lesion of syphilis." When, however, we exclude the large number of cases in which the father is already syphilitic, and remember that in the others the contact between him and the child is slight, infrequent, and fleeting—being usually limited to an occasional kiss or caress—it does not seem strange that no instances of such infection have been reported; nor does the fact seem at all worthy of being made the foundation of a general law.

There can be but one rational explanation of these facts—viz. that the mothers who have thus acquired an immunity have done so by first acquiring the disease. It may, in those cases where no secondary symptoms appear, be in a modified form, due, as suggested by Mr. Hutchinson, to some heteromorphism or alternation of generations on the part of a specific fungus, or to the excessively small quantity of the poison which finds its way from child to mother, or to some other obscure cause with which we need not now concern ourselves. It is, at any rate, no more mysterious than the protective influence of vaccine in small-pox, and when one is rationally explained doubtless the other will be found to be closely allied in its mode of action. The fact which interests us at present is that it is in the highest degree improbable that anything but some form of syphilis itself could afford this entire protection, and that it is not in the least unreasonable, but, on the contrary, logical and consistent with all the known facts, to suppose that while in some cases no observable symptoms might be produced, in others where the cause was the same, but more active, or the powers of resistance less, the usual constitutional phenomena would be developed.

We may conclude, then, that the husband may infect his wife—(1) In the usual manner or by direct contagion; (2) through the medium of the child, or at any rate by the production of conception.

The theory upheld by Von Baerensprung, that the syphilis of the mother is imparted to her at the moment of impregnation, the disease being impressed upon the fecundated ovule, does not materially conflict with the above views, conception being in either case the essential factor, but in the latter instance the intervention of the foetus itself not being necessary. It has to support it the clinical fact that in those cases where syphilis appears during pregnancy the outbreak of symptoms occurs at about the ninth or tenth week after the date of conception, or a period which closely corresponds to that of the appearance of general symptoms after exposure to ordinary contagion—allowing about three weeks for the so-called incubation of the chancre and six weeks for the secondary incubation.³²

³² This has been shown not only by Von Baerensprung (Die Hereditäre Syphilis), but also by Diday, whose observations were intended to prove the possibility of syphilis being derived from the child by the mother—"choc en retour." In 24 cases the period at which the first eruption appeared in the mother averaged sixty-five days after conception; only once did the first signs appear after the fourth month of pregnancy.

It may also be said to be rendered probable by the following line of argument:

Colles's law, which is without exception, demonstrates that every woman who has had a syphilitic child has been herself infected, even if she has had no observable symptoms;³³

Cases are recorded, however, in which a woman having given birth to one or more syphilitic children, and therefore herself syphilitic, bears healthy ones in consequence of specific treatment administered to the father before and during the period of conception, she remaining untreated;³⁴

The determining cause, therefore, of the syphilis of the child is not the syphilis of the mother, but the condition of the fecundating germ of the father; and, as a corollary,

The determining cause of the syphilis of mothers in whom the disease follows conception is not by infection from the foetus through the utero-placental circulation³⁵ or otherwise, but is the diseased male procreative cell which becomes blended with the female ovule.³⁶

³³ It is obviously no explanation of the law of Colles to say that "it would seem to indicate that the escape of the mother is due to some occult, undiscernible change in her system" (Bumstead and Taylor, op. cit., p. 745).

³⁴ See foot-note, p. 262.

³⁵ On account of the absence of cellular elements in the fluid interchanged.

³⁶ An elaborate paper by Fraenkel (Archiv für Gynaekologie, 1873, vol. v. p. 1), based on twenty-one cases of childbirth, was written to prove by the condition of the placenta that direct infection of the child by the father was possible without the participation of the mother, and that when the latter became infected it was through the medium of the child (choc en retour). In fourteen of his cases the specific change in the placenta began in the foetal portion or affected it exclusively. These cases, however, as analyzed by Hill and Cooper (op. cit., p. 57), are altogether defective in important particulars. Of course to sustain his theory there should be indubitable evidence that the fathers were syphilitic, and that the mothers were not so. So far from this being the case, but one of the fathers was known to be syphilitic, while two of the mothers had evidences of constitutional syphilis at the date of childbirth, and nine others had markedly suspicious histories. The lesions of the placenta which are thought to be syphilitic consist in the development of papular or gummatous growths which give rise secondarily to inflammatory troubles affecting either the placenta itself or the placenta and the uterine mucous membrane.

If the premises are admitted the conclusions seem irresistibly to follow.

There is no proof whatever that the semen of a syphilitic man is contagious or can transmit the disease in any but the way above discussed. On the contrary, it has been shown experimentally³⁷ that it is entirely innocuous and non-inoculable.

³⁷ Mireur, Annales de Derm. et Syph., 1876, p. 77.

All other theories as to methods of contagion are so entirely hypothetical and unsupported by trustworthy evidence that we can afford to disregard them.

We may now consider the ways by which syphilis reaches the child, and they may be broadly classified into—

1. By descent from the father.

2. By descent from the mother.

3. By direct infection.

As a matter of course, the influence of the father upon the child, so far as regards heredity, ceases at the moment of conception; or, to be more exact, no subsequent condition of the male parent, no development or acquirement of disease, can exert any further effect. That the existence of active syphilis in the father may result in the transmission of the malady to the child can hardly be doubted. To be sure, there are numerous examples of cases where both wife and child have escaped though the husband showed at the time of conception very active secondary lesions. These, however, are exceptions, and do not in the least invalidate the rule that it is possible for a father to hand the disease directly to his child. There is no other possible interpretation of the cases already alluded to in which successive pregnancies in the same woman alternately resulted in healthy or in syphilitic children according as the father was or was not kept under specific treatment. Other arguments might be advanced, but that seems to me conclusive.

The relative effect of paternal as compared with maternal influence may be considered after we have described the latter.

Descent from the mother may occur theoretically in consequence of—

1. Infection of the mother previous to conception.

2. Infection of the mother at the moment of conception.

3. Infection of the mother during the period of utero-gestation.

As to the first of these methods of transmitting the disease there is little if any difference of opinion. Even those who claim the most for paternal influence³⁸ include among the conditions which may give rise to syphilis in the child disease of the ovule, and it may be stated as incontrovertible that recent or active syphilis in the mother at the time of conception will almost certainly be followed by syphilis in the child. As a rule, women who have borne syphilitic children, even when they do not give unmistakable evidence of the disease, fail in health, become anæmic, and often develop glandular or osseous swellings which, according to Zeissl,³⁹ are only relieved by antisyphilitic treatment. The bearing of Colles's law upon the alleged immunity in many instances of the mothers of syphilitic children has already been shown ([p. 264]); and there is other evidence, not perhaps so conclusive, but strongly corroborative, of the same view—viz. that their escape is only apparent, and that syphilis, either latent or active, always affects such mothers.⁴⁰ There can be no manner of doubt that in every instance at or about the time of childbirth there are not to be found pathognomonic lesions of syphilis, nor do such lesions always make their appearance in cases where the period of observation is a short one; but it is claimed with much show of truth that prolonged and patient inspection of such patients will in time result in the discovery of some symptom which betrays the presence of the disease.

³⁸ Bumstead and Taylor, op. cit., p. 745. Hutchinson in Reynolds's System of Medicine, Am. ed., vol. i. p. 431: "In a large proportion of the cases met with in practice the taint is derived from the father only." (On this point see foot-note to [p. 270].) If there were any doubt as to the fact that syphilis in the mother only may be transmitted to the child, it would be removed by the cases of Bardicet and others mentioned in Nouv. Dict. de Méd. et Chir., vol. xxxiv. p. 688. In these cases nurses who had contracted the disease from their sucklings subsequently became pregnant (without having infected their husbands) and gave birth to syphilitic children.

³⁹ Jahrbuch, vol. ii. p. 303, 1872.

⁴⁰ Zeissl, Sigmund, Oewre, Flindt, Woodman, and others are quoted by Hill and Cooper to this effect. Woodman, for other purposes, gave the histories of 200 cases of infantile syphilis. In all of these the mothers had suffered from typical secondary lesions.

The cases in which treatment of the father has resulted in healthy children, whereas without treatment he procreated only syphilitic children, the mother being without either symptom or treatment, have been urged as evidence of the direct descent of syphilis from the father to the child without the intervention or participation of the mother. Doubt has been thrown upon them by those who uphold the contrary view;⁴¹ but after looking into them carefully I am constrained to admit that some of them, notably those of Kassowitz⁴² and R. W. Taylor,⁴³ are convincing of the facts—(1) that treatment of the father controlled the condition of the child, and (2) that there was no evidence that the mother had syphilis. But we have seen that the only proof of the universal infection of mothers of syphilitic children is the law of Colles, and that in many cases the disease is for a long time latent or unrecognizable. Let us admit that this was the condition in the cases in question; it does not at all follow, necessarily, that because the mother has latent or hidden syphilis she must infect her child. Every case even of active syphilis in the parents is not handed down to the children, a certain proportion of whom escape even when both parents are in the height of the secondary stage at the time of conception.⁴⁴ But the activity of the disease in the children, and even more the likelihood of its reaching them, are in direct proportion to its activity in the parents.⁴⁵ A mother, therefore, who under the influence of active syphilis in her husband has given birth to two or three syphilitic children, and has herself shown no symptoms of the disease, may nevertheless have it in the latent form and have no strong tendency to transmit it. Consequently, treatment of the father will result in the procreation of healthy children, because it removes the active and efficient cause of their infection. To take any other view of these cases is to assume that every syphilitic parent must hand down the disease to the children—an assumption which is not in consonance with numerous well-attested clinical facts.

⁴¹ Hill and Cooper, op. cit., pp. 52, 53.

⁴² "Die Vererbung der Syphilis," Stricker's Med. Jahrb., 1875, p. 391.

⁴³ Archives of Clinical Surgery, New York, Sept., 1876.

⁴⁴ Fournier, op. cit., pp. 35-37.

⁴⁵ Hutchinson, op. cit., p. 431.

Leaving this interesting question, however, we may consider the other methods by which syphilis descends from the mother to the child, having seen already that it is beyond doubt that it may be handed down by disease of the ovule due to syphilis acquired previous to conception, and having seen that there is a high degree of probability that the mother herself rarely, if ever, escapes the disease. The second method, or that in which the mother becomes syphilitic at the moment of conception, has already been sufficiently discussed. It is really, strictly speaking, an example of paternal heredity, as the resulting germ is syphilitic—not because the ovule of the mother was infected, but on account of the disease of the spermatozoid of the father.

There remains for consideration the influence upon the child of a syphilis acquired by the mother during some period of utero-gestation. That under these circumstances the child can become infected has been and is still absolutely denied by some very respectable authorities.⁴⁶ All that is necessary for proof of its occurrence is, however, (1) freedom of both parents from syphilis at the time of conception, or, in other words, syphilis must have been acquired by both—not alone by the mother—after the beginning of pregnancy; (2) that the syphilis of the child be unmistakably pre-natal—that is, not acquired by some accident during or after birth.

⁴⁶ Bumstead and Taylor, op. cit., pp. 742, 744. They base their denial, first, on the physiological fact (?) that no interchange of cellular elements between mother and father is possible, and next on the absence of satisfactory evidence of the occurrence of infection during pregnancy. Zeissl's case seems sufficient answer to the last assertion, and there is strong evidence that the first is without good foundation. "The placenta is penetrated by the virus, and does not play the part of a filter for the elementary particles of matter which, so far as we know, represent the true active contagion of the disease. In the absence of direct experiments, which it would be almost impossible to institute, we may argue from the facts known to exist in certain acute infectious diseases in which there are very interesting points of resemblance.

"It has been known for a long time that small-pox occurring in the mother may be transmitted to the product of conception enclosed in the uterus, and it is supposed that the virulent particles traverse the walls of the maternal vessels in order to penetrate the circulating apparatus of the foetus. But what is only a supposition in the case of small-pox seems to have been actually demonstrated in symptomatic charbon (Arloing, Cornevin, and Thomas), bacteridian charbon, and recurrent fever, in which the poisonous element is easily recognized. The recent experiences of Strauss and Chamberland (1882) have shown that the foetus participates in the 'infection charbonneux' of the mother. Albrecht has shown (1880) the presence of numerous spirochoetæ in the blood of the heart of a child born at seven months of a woman with a second attack of relapsing fever. It may be supposed, therefore, though not demonstrated, that the transmission of syphilis takes place by the same method as that of relapsing typhus or of charbon" (Nouveau Dict. de Méd. et de Chir., pp. 682, 683).

The following case,⁴⁷ reported by a most accurate observer, seems to combine both these requisites. Zeissl the younger reports that O. X., thirty-six years old, never having had syphilis, left his wife, to whom he had been married two years, to go a journey on July 15, 1877. The wife was then in the second month of her first pregnancy. On July 24th O. X. had extra-marital intercourse. About twenty-one days after this coitus he observed a small lump on the inner surface of the foreskin, and on Aug. 22d he consulted Zeissl the elder. On Sept. 23d a maculo-papular eruption of the skin with erythema faucium appeared. Under treatment these symptoms completely disappeared. On Oct. 29th he went home to fetch his wife to Vienna for her lying-in, and had intercourse with her soon after his return, notwithstanding Zeissl's strict prohibition. At the beginning of December a hard sore developed on the left nympha of the wife, who was then in the seventh calendar month of her pregnancy. At the end of December a maculo-papular eruption spread over the body and was treated with mercury. On Feb. 14, 1878, a well-grown and apparently healthy female child was born at full term. When eleven days old⁴⁸ a pustulo-scaly eruption came out on the child's soles and toes, and soon afterward a maculo-papular eruption over the body generally. A few days later the child died. No post-mortem examination was permitted. In July, 1878, the wife had iritis, and after that gummata on the leg. She miscarried in July, 1878, at the third month, and again in February, 1879, at the second month.

⁴⁷ Quoted by Hill and Cooper, op. cit., p. 60.

⁴⁸ Of course much too early for constitutional symptoms if the disease had been acquired during or after birth.

There seems to be no reasonable escape, after reading this carefully, from the conclusion that in some manner the poison of syphilis found its way from the mother to the child. The old idea that the latter was directly infected in utero from the semen of the father is altogether without foundation. Other cases equally satisfactory and complete have been reported, and, unless the intelligence or the truthfulness of the observers be impugned, establish without doubt the possibility of infection during utero-gestation.

In the above case the contagion of the mother occurred in the seventh month of pregnancy; and this, I believe, is as late as it has ever been known to be communicated to the child. The exact date at which it becomes impossible so to transmit it is unknown, but as a general rule it may be said that the earlier a mother is infected during gestation the less likely is it that the child will escape. Treatment of the mother—as of the father in cases where he is at fault—very greatly modifies the whole problem and adds immensely to the chances that the child will not be infected.

Direct infection of the child during birth could not properly come under the head of hereditary syphilis. There is no possible reason why, when the mother has contagious lesions of the genitals, acquired too late to infect the child in utero, this should not occur, but as a matter of fact no such case has ever been recorded. One explanation of this circumstance may be found in the protective covering of vernix and mucus which coats the infant's body and lessens greatly the risk of absorption. This hardly accounts satisfactorily, however, for the entire absence of such cases from medical literature, and it is fair to suppose that in all but those cases in which the primary sore is acquired during the last month of gestation—which for obvious reasons are excessively rare—the infant acquires some immunity which protects it from its mother, and is similar to that which, under Colles's law, operates in her favor. In other words, even though apparently free from syphilis at birth—a not uncommon event, as we shall see—it has a latent or modified syphilis which protects it from contagion.

We may now briefly restate the conclusions at which we have thus far arrived:

1. After a certain interval, not less than four years, and after thorough specific treatment, a person who has contracted a syphilis not especially severe or malignant in its type may be permitted to marry. The assent to marriage will then be based on a belief in the curability of syphilis or the cessation of its contagiousness, its inoculability, and, in the vast majority of cases, its transmissive power at the end of the secondary stage.

2. It may be inherited from either parent or from both, and the probability that this will occur increases in a direct ratio with the nearness of the time of conception to the date of their infection with the disease. The severity of the inherited disease in the child increases in the same proportion.

3. It is undoubted that, the father being healthy and the mother syphilitic, the child may, and in all probability will, have the disease.⁴⁹

⁴⁹ Dr. Sturgis, who disbelieves altogether in the possibility of paternal heredity, concludes, after examining the subject carefully, that (1) a mother begets non-syphilitic children as long as she is not infected, even though the father is syphilitic; and (2) the moment she is diseased the children are inevitably so (Paper on "The Etiology of Hereditary Syphilis," New York Medical Journal, July, 1871). This doctrine was previously supported by M. Cullerier, whose views gave rise to the remarks of M. Voillemier (quoted by Fournier) that if they were accepted "the father would be only the accidental occasion of a child; one would be, in reality, the child of his mother only." Cullerier's cases are invalidated by the fact that the syphilitic fathers who had healthy children had been subjected to mercurial treatment (Mém. de la Société de Chirurgie, Paris, 1854, quoted by Taylor in Archives of Clin. Surg., vol. i. p. 83). The theory is a very old one. Vassal has sustained this idea as long ago as the end of the last century. Kostum (1804), and after him Hufeland, were of the same opinion. Cullerier (1857) wrote: "In order that a child acquire syphilis hereditarily it is necessary that the mother is or has been syphilitic." Notta, Follin, Charrier, Mireur (1867), and Langlebert (1873) support this theory more or less earnestly. Oewre wrote (1873): "Paternal influence is nil as regards hereditary syphilis." Isseff (1879) wrote: "Where a man suffers or has suffered from syphilis he cannot transmit the disease to his descendants without infecting his wife; that is to say, in fewer words, there is no infection from the father." Sigmund says: "The heredity of syphilis is derived in its last analysis from the mother" (Nouveau Dict. de Méd. et Chir., vol. xxxiv. p. 689).

4. It is probable, but less so,⁵⁰ that, the mother being healthy and the father syphilitic, the child will be infected.

⁵⁰ This refers simply to the comparative probability of infection, and does not conflict with the statistical fact expressed by Hutchinson (Reynolds's System of Medicine, vol. i. p. 431) in his words: "In the large proportion of cases met with in practice the taint is derived from the father only." This numerical predominance of paternal influence is very readily explained. There are many more syphilitic men than syphilitic women, and especially among the couples who contract fertile marriages the number of women who are infected before becoming mothers is inconsiderable. On the other hand, it frequently happens that men who have had syphilis, but have been without symptoms for a longer or shorter interval, marry and transmit to a series of children a disease which has ceased to be directly contagious to their wives, the transmissive power continuing after the possibility of ordinary contagion has disappeared. As in the majority of such women the disease is latent, and may be only displayed in their immunity from infection, it becomes evident that, history and symptoms both being wanting on their part, the conditions justify the assertion of Mr. Hutchinson. (See Nouveau Dict. de Médecine et Chirurgie, p. 684.)

That assertion (quoted above) has, however, been thought by several writers to indicate his belief in the escape of the mother. That I have not misinterpreted him is evident from the following extract from an article on "The Transmission of Syphilis," written by him (Brit. and For. Med.-Chir. Rev., Oct., 1877): "I take it for granted (although I know that there are still some who doubt) that it is possible for a father to transmit the taint, the mother being at the time of conception wholly free. I believe, indeed, that in practice this is by far the most common way in which syphilis is transmitted. Whether in these cases it is correct to speak of the inheritance being paternal only is, as we have just seen, another matter, since it is possible that in every instance the mother derives an infection from the father, and may thus in turn influence it."

5. It is highly probable, though it can hardly be considered as proven, that in all cases where a child becomes syphilitic through paternal influence the mother is also the subject of syphilis, which may, however, assume a latent form, the only evidence of its presence in a few cases being the protection which it affords against contagion through the medium of the child.

6. Syphilis may be transmitted from mother to child even when it is acquired by the former as late as the seventh month of utero-gestation.

Since writing the above the thirty-fourth volume of the Nouveau Dictionnaire de Médecine et de Chirurgie has been published. In the article on syphilis seventeen pages are devoted to the question of heredity, which is reviewed in a most thorough manner and finally summed up as follows (p. 698):

"The most definite views which we possess on the subject of the hereditary transmission of syphilis may be thus expressed:

"Children may be infected by heredity, not only when the two parents are syphilitic, but also when only one, either the father or mother, is diseased at the time of conception.

"When both parents are diseased at that time there is more certainty that the child will be infected, and infected gravely, than if only one of them has the pox.

"The hereditary disease is not always fatal, even when both progenitors have actual specific symptoms. The more recent the disease of the parents the greater the chances of their transmitting the disease and of its assuming a serious form. There is no proof that inherited syphilis is more grave when derived from the father than when coming from the mother.

"It is altogether exceptional for the mother, healthy at the moment of conception, not to participate in the disease transmitted by the father to the child. If she escapes direct contagion—which is rare when the disease of the father is active—she undergoes a species of infection from contact with the contaminated foetus.

"Syphilis by conception, which is thus transmitted from the foetus to the mother, may present the usual characters and evolutions of the acquired disease; frequently, also, it is latent, and is betrayed only by the existence of immunity from further contagion on the part of the mother. It may finally manifest itself by tertiary symptoms or by systemic troubles without specific characters.⁵¹

⁵¹ "This form of syphilis shows itself in the mother in three ways:

"1st. By the usual signs of syphilis by contagion, with the exception of the primary sore, appearing about the sixty-fifth day after conception.

"2d. Appearing at a later date as secondary or even as tertiary symptoms, and preceded merely by a little disturbance of the general health, unhealthy appearance of skin, falling of hair, etc., but nothing truly specific.

"3d. Showing itself some years afterward in a tertiary form, having in the mean while given no indication of its existence save only in the protection it afforded against contagion from the child" (Nouveau Dict. de Méd. et Chir., vol. xxxiv. p. 696).

"The power of transmitting syphilis hereditarily decreases spontaneously as the disease of the parent becomes older. The influence of treatment is no less certain than that of time. When the two progenitors are at the moment of conception free from syphilis, the foetus may still be infected if the mother acquire the disease during her pregnancy."

Syphilis of the placenta is of especial interest in its relation to the abortions and stillbirths so frequent in syphilis. Until the elaborate paper of Fraenkel upon this subject (see foot-note, [p. 265]) almost nothing was known about it. He describes⁵² the macroscopic changes as consisting of increased size and weight of the placenta, closer and firmer texture of the placental tissue, the presence of old and recent extravasations of blood in all stages, opacity and thickening of the decidual covering and of the amnion and chorion, which are in places adherent to each other. Microscopically, it was found that the placental villi were filled with small nucleated cells, which were especially abundant in the centre of the villous spaces along the axis where the vessels usually take their course. The ends of the villi were enlarged with knob-like processes.

⁵² I condense here from the translation of Fraenkel's paper, which constitutes the bulk of chap. xxvii. of Bumstead and Taylor's excellent work.

Fraenkel's explanation of these changes is as follows: Under the influence of syphilis⁵³ cell-proliferation begins in the villi, which are, normally, only sparingly supplied with cells. These new cells excite proliferation of the cells of the connective-tissue stroma and of the epithelium. This proceeds to such an extent that it leads to compression of the vessels,⁵⁴ interfering with the circulation, and finally obliterates them. The vascular spaces into which the villi dip become filled up and narrowed, and often disappear. In this way, and also by reason of the thickening of the epithelium, the interchange between the maternal and foetal blood is interfered with, and at last is prevented.

⁵³ Fraenkel took as his criterion of syphilis the presence of the osteo-chondritis described by Wegner (see [p. 286]).

⁵⁴ "Hennig was the first who called attention to the intimate relation of the cell-growths to the vessels. The obliteration or compression of numerous vessels of the villi interferes with the mutual interchange of gases between the maternal and foetal blood, causing fatty degeneration of the villi and, if the process is extensive, the death of the foetus" (article "Syphilis," Ziemssen's Cyclopædia, vol. iii. p. 237).

If this process is spread over the whole placenta, the foetus perishes before it is complete. If it is limited to circumscribed areas, it may continue to live for a shorter or longer period.

These observations require to be repeated and confirmed, but they have served to open up a most interesting branch of syphilitic pathology.

Syphilis in the parents will manifest itself in the children in one of several ways, which are determined chiefly by two factors—viz. first, the length of the interval between the infection of the parent and the date of conception; and, second, the thoroughness of the treatment of the parents during that interval. To these may be added as subsidiary, but still of definite importance, a third, the type of disease which has affected the father or mother, whether mild or severe, benign or malignant.

From what has already been said in reference to the question of marriage, it will be at once understood that the danger to the offspring in untreated cases, and in those where conception has occurred during the early secondary period of the disease, is of the most extreme gravity.⁵⁵ In such cases the usual result of pregnancy is abortion at from the first to the fifth or sixth month,⁵⁶ the foetus sometimes exhibiting the evidences of the disease in the shape of large bullæ upon the palms and soles, or in the presence of characteristic visceral lesions, but quite as often showing nothing distinctive. It has generally undergone more or less maceration, and the skin, which is readily detachable, is of a congested, purplish color.

⁵⁵ According to Kassowitz, in women who are not treated all pregnancies occurring within the first three years of their infection terminate either in abortion or in the birth of children who survive for only a few weeks or months.

Weber (quoted by Parrot, Le Progrès Médicale, Nov. 24, 1877, p. 882) treated thirty-five pregnant women by mercurial inunction, and they all went to full term under normal conditions. Among those treated with mercury and iodide of potassium, but who, by reason of intolerance of the former drug, took chiefly the latter, 20 per cent. aborted; when the mixed treatment was carried out regularly 15 per cent. aborted; and when only iodide of potassium was given 36 per cent. aborted. His studies were made on 109 syphilitic pregnant women. Parrot himself says (ibid.) that "abortion occurs in about one-third of all syphilitic women. Syphilis should always be suspected when this accident occurs several times successively." "The date of abortion depends on the age of the syphilis of the woman. It is most to be feared during the evolution of secondary symptoms, particularly when they are grave. There is more probability that it will occur when infection has preceded pregnancy than when it occurs during its course. Nothing is so much to be feared as contamination of the ovule. The nearer the date of infection of the mother approaches to full term, the fewer the chances of abortion. It is not likely to occur even when the mother is infected at five months."

⁵⁶ Many observers think that abortion results directly from the death of the foetus. Babington (notes to Hunter's Treatise on Venereal), Trousseau, and Von Baerensprung were of this opinion.

Dr. Cory thinks that in many cases it is possible that the effect of syphilis may be to effect so early an abortion that the case is simply regarded as one of delayed menstruation or of menorrhagia. Such a conception would, however, be competent to infect the mother, and might seem to explain cases otherwise involved in obscurity.⁵⁷ At least one-third of all syphilitic children are dead born.⁵⁸ As time goes on, however, and the intensity of the parental disease is lessened, or in cases where appropriate treatment has been applied, either the abortion occurs at a later period of pregnancy or the children are brought alive into the world. Even then, however, and although at birth they may show no evidences of the disease, their chance of escape is but small. One-fourth of them die within the first six months. If they survive that period, the chances for life are slightly in their favor, but those for health or freedom from deformity and disease are still overwhelmingly against them.

⁵⁷ As, for instance, when a woman married to a syphilitic man, but without issue, remarries a man with no history of syphilis, and yet gives birth to a syphilitic child. In such a case there would be no history of direct infection and none of pregnancy, the only two modes by which she could have contracted the disease, and the father—the second husband—might be unjustly suspected.

⁵⁸ Kassowitz, op. cit.

The course of inherited syphilis differs strikingly from that of the acquired disease. It will hardly be necessary to do more than remind the reader of the ordinary stages of the latter affection—the primary, which includes the period of the chancre and of lymphatic engorgement, lasting about sixty to seventy days; the secondary, or exanthematic, characterized by copious cutaneous eruptions and extensive involvement of mucous surfaces, lasting from one to three years; the intermediate,⁵⁹ or the stage of latency and relapses, lasting for a very variable period, from three to ten years, but under proper treatment very much reduced or altogether abolished; and finally, the tertiary period, beginning four or five years after contagion, extending indefinitely throughout life, but often in cases properly treated absent altogether.

⁵⁹ So designated by Mr. Hutchinson, and for clinical purposes a very valuable addition to the periods of syphilis. He describes it as follows: The patient may be either wholly free from symptoms and in good health, or he may remain pale and rather feeble, and liable from time to time to slight returns of eruption on the skin, sores on the mucous membranes, condylomata, etc. He is protected as regards fresh contagion, and should he beget children they are almost certain to suffer. The relapses during this stage are usually easy to be distinguished from true secondary symptoms. There is little or no febrile disturbance, the rash is not copious, and often not symmetrical. Acute iritis, retinitis, etc. never occur for the first time, though they may do so in the form of relapses.

For purposes of description and of contrast we may similarly divide the whole period of evolution of a case of inherited syphilis,⁶⁰ omitting the primary stage, which has never been found to exist in true cases of hereditary syphilis. Of course in congenital or infantile syphilis, in which by direct contagion, either from the mother or from any one else, the disease was acquired by the child, the course would not differ materially from that observed in the adult. But as this stage in all probability corresponds to the period during which the poison is already finding its way into the system through the lymphatics, of course it is not found in the child who is infected from the moment of conception or who receives the poison from the mother directly into the circulation.⁶¹ For from one to three weeks the infants often show no symptoms of the disease. In 158 cases collected by Diday, 86 manifested symptoms of the disease before the expiration of the first month, and 60 of the remainder before the end of the third month.⁶² When to these are added the statistics of Roger, we find that of a total of 172 cases, 159 showed syphilitic symptoms before the end of the third month.⁶³ When the symptoms are present at birth, they consist largely in a general withered, atrophied, weazened appearance of the child; a hoarse cry, due to swelling, with subacute inflammation, or even ulceration, of the laryngeal mucous membrane; a coryza,⁶⁴ due to a similar condition of the Schneiderian membrane; and certain cutaneous eruptions, the most common of which at this early date is the large vesicular or bullous eruption known as syphilitic pemphigus.

⁶⁰ The idea that the character of the symptoms which first appeared depended upon and corresponded with the stage of the disease in the parent has now, I believe, no supporters. It was once thought, at least by some syphilographers, that if the parents were in the tertiary stage at the time of conception the child would develop tertiary symptoms, omitting the other stages.

⁶¹ If chancre were the first symptom of constitutional syphilis, why should it not appear in cases of hereditary syphilis?

⁶² Infantile Syphilis, p. 101

⁶³ About 1823, from 16,000 to 17,000 children were admitted annually to the wards devoted to foundlings at Lyons. Clièt, recording his experience with this disease, says that syphilis is one of the most common of their maladies, but that "it exceedingly seldom shows itself at birth by evident signs" (Compte-Rendu Méd. Chir. des Observations à l'Hôpital général de la Charité de Lyon, 1823). Cristoferi, physician to the foundling hospital at Bologna, says that syphilis generally manifests itself between the ages of one and three months. Never once, he states, was a newly-born infant admitted with the disease unquestionably developed (Gazette Medica di Milano, 1844). Trousseau says that it "rarely appears before the second week, and very exceptionally after the eighth month" (Lectures on Clin. Med., vol. iv. p. 331, London, 1871, ed. of New Sydenham Society).

⁶⁴ Usually appears later, but exceptionally at birth.

Pemphigus.—With regard to the specific or non-specific character of this eruption there has been much difference of opinion, and, as it is often the earliest distinctive expression of syphilis, a diagnosis of which could hardly be founded on the general appearance of the child, or even on the hoarse cry and the coryza, it becomes important to have definite ideas upon the subject. Nearly a century ago (in 1794) it was denied⁶⁵ that this eruption was a manifestation of venereal disease; and this view has been supported by many able and accurate observers down to the present day. In 1851 a discussion upon the subject took place in the French Academy of Medicine, which elicited the opinions of the majority of those members who were entitled to speak with authority in the matter, and which has since been referred to by most writers. Cazeaux upheld the non-syphilitic hypothesis on the ground (1st) that the so-called syphilitic pemphigus of children does not differ from the simple pemphigus of adults, presenting none of the physical characters which distinguish the specific cutaneous eruptions; (2d) that it appears at birth or immediately after, while the symptoms of hereditary syphilis generally show themselves later;⁶⁶ and (3d) that at that time there had been seen no cases of pemphigus at the Lourcine Hospital, where so many syphilitic children were born.⁶⁷ In this view he was supported by Trousseau, Lasègue, Gibert, Bazin, and other obstetricians and syphilographers of note.⁶⁸

⁶⁵ Ariander, Mémoires de Méd. et d'Accouch., quoted by Diday.

⁶⁶ This is now known to be an unreliable distinction, expressing perhaps a general rule, but one with so many exceptions as to render it void of diagnostic significance.

⁶⁷ This may have been true at that time, but has certainly not continued to be so. Cornil says: "We often see at the Lourcine children born prematurely or at the full term with pemphigus, either fully developed at the moment of birth or appearing a few days afterward, and who commonly die with syphilitic cachexia, the sad heritage derived from their maternal parents."

⁶⁸ Pemphigus may indeed be a specific affection, but no characteristic sign has been discovered sufficient to distinguish it from the ordinary form of pemphigus. "On the other hand, there is no inconsistency in admitting that syphilis, which so deeply impairs the constitution of the parent, may act like any other common cause and excite non-specific pemphigus; for an infant is badly lodged and poorly nourished in the womb of an enfeebled mother, apart from the influence of the virus" (Ricord, note to John Hunter's Works, 1853).

On the other hand, Dubois claimed a specific character for the affection on the ground (1st) of the fact that in the majority of cases there was a syphilitic history in the parents; and (2d) that the eruption often coexists with well-known syphilitic lesions. This was supported by Cazenave, Danyan, Bouchut, Vidal, Ollivier, and others.⁶⁹ Diday, who devotes several pages of his interesting work on Infantile Syphilis to this subject, regards the eruption as simply a manifestation of a cachexia produced by syphilis,⁷⁰ founding this opinion on (1st) the absence of specific characters in the eruption; and (2d) that syphilitic pemphigus is a rare affection in the adult, if it occurs at all, so that to recognize it in the child would be to make a single exception to the general rule that "all the syphilitic eruptions of new-born children have their equivalents in those of adults." He explained the two cases which were then (1858) recorded of cures of pemphigus by mercury⁷¹ by saying that it was the treatment of the diathesis, not of the disease, which caused the improvement. He acknowledges, however, the very frequent association of pemphigus in the child with syphilis in the parent, and says that it springs from the latter affection, "specially, but not specifically"—a rather wire-drawn distinction.⁷²

⁶⁹ Jullien (op. cit., p. 1005), after considering the opposing views as to the character of this eruption, says: "We have no hesitation in declaring ourselves in accord with Roger, Ollivier, Ranvier, Parrot, and others, and in distinctly separating from the specific affection the rare eruption known as simple pemphigus, sometimes epidemic, occasionally febrile, and appearing most frequently about three months after birth. We consider likewise that an evidence of congenital syphilis which is by no means doubtful is found in the bullous eruption seen at birth or within the first two weeks, comparatively frequent, and involving by preference the palms and soles. This opinion is based upon (1st) its appearance in children whose parents are known to be syphilitic; (2d) its association with syphilitic lesions of the lungs, liver, kidneys, thymus gland, etc.; (3d) its partial disappearance under mercurial treatment, and its reappearance when that treatment is discontinued."

⁷⁰ Op. cit., pp. 70-77.

⁷¹ Depaul, Gaz. Méd. de Paris, 1851, p. 472, and Galligo, Gaz. Med. Toscana, 1852, p. 123.

⁷² Trousseau (Clinical Lecture on Syphilis in Infants), after detailing a case in which there was some doubt as to the existence of hereditary syphilis in a child born alive, and in which case the previous pregnancy had resulted in a stillborn child at seven months, the body of the latter having been preserved in alcohol and exhibiting numerous traces of pemphigus, says: "So far as I was concerned, this demonstration did not amount to more than the establishing of a probability, and several physicians who participated in this indecision finally accepted a compromise. They considered that maternal syphilis had determined a sort of cachexia in the foetus which had led to an eruption of bullæ which was not specific. By accepting this too-facile hypothesis you will imprudently open a door which you will with difficulty be able to close."

As these differences of opinion have been perpetuated to the present day, it has seemed to me proper to make this reference to their history, although I am strongly convinced that the progress of clinical and pathological knowledge enables us now to assert that although, as an exception, bullæ may be due to a profound cachexia not dependent on syphilis, yet that in the large majority of cases they are specific in their character.

The argument which always seemed to me the strongest, the fact that a similar eruption is almost—or quite—unknown in the adult, has been removed by the observations of Cornil, who has shown that it belongs properly with the papular rather than with the bullous eruptions, and should be classed with the roseola and papules of early syphilis—just where, from its clinical history, we should expect to find it. The raising of the epidermic layers is due chiefly to their delicacy, their slight resistance, and their previous immersion in the amniotic fluid—i.e. to conditions which are peculiar to the skin shortly after birth.⁷³ He founds these very important opinions upon the autopsy of a child stillborn a little before full term, the mother being in the height of secondary syphilis. The child presented characteristic bullæ on the soles and palms. After hardening these were found to consist of the two layers of epidermis placed one above the other. Fig. 6 represents a bulla about one centimeter in diameter which was situated on the plantar surface of the great toe.

⁷³ Cornil, op. cit., p. 203.

FIG. 6.

Pemphigus bulla from a new-born syphilitic child. The superficial epidermic layer e is elevated by a fluid exuded between it and the rete mucosum. The rete mucosum, c, is also partly raised, so that there exists a space filled with fluid between it and the papillæ, p. The epithelial prolongations and the ducts of the sudorific glands m, placed between the papillæ, and which run between them into the derm, are broken and suspended from the rete mucosum. d. Derm. a. Fibrous and muscular layers. t. Tendons and fibrous tissue. o. Cartilage of ossification of the first phalanx. v. Vessels. X 8.

FIG. 7.

Section of the rete mucosum and papillæ from the same case of pemphigus as Fig. 6. o. Orifice of a sudorific gland. m. Cells of the rete mucosum, some of which are excavated, c. p. Papillæ. v. Their vessels. n. Prolongations of the rete mucosum between the papillæ. X 200.

If, then, we find an infant at birth or immediately after⁷⁴ presenting on the soles, the palms, the fingers and toes, or on the limbs, an eruption consisting of blebs more or less perfectly distended with a liquid which may be clear, cloudy, or bloody, circular or oval in shape, sometimes irregular, seated on inflamed, reddish skin, and surrounded by trifling areolæ, we may strongly suspect the presence of syphilis in an active and most menacing form. And this suspicion becomes a certainty if, in combination with such an eruption, the general cutaneous surface is yellowish or muddy in hue, is hard, dry, wrinkled, without elasticity or softness—owing to the absence of subcutaneous fat—and, for the same reason, is furrowed and wrinkled about the face, imparting an appearance of senility; if the child has a hoarse cry, a discharge from the nostrils; and, of course, if there are at the same time other and unmistakable syphilodermata. This eruption is specially important, however, because upon the recognition of its specific character in cases of stillbirth, or in those in which the child survives only a few days—not long enough for the development of further symptoms—will depend the opinion as to the cause of death, which, whether expressed or not, will determine the future treatment of both parents during the interval and of the mother during the next pregnancy.

⁷⁴ Non-syphilitic pemphigus is said to be never present at birth, nor until the child has become considerably exhausted by wasting from some defect of nutrition. It therefore does not appear until it is several weeks old. It then attacks the trunk in preference to the palms and soles.

We may now consider the other symptoms of the secondary period in the child.

Coryza is one of the most characteristic, and at the same time one of the most important, of these in its influence on the health of the child. It is due to the same condition of the mucous membrane lining the nasal fossæ as manifests itself simultaneously or soon afterward on the skin in the shape of erythema, roseola, or papules; in other words, it is a hyperæmia with papillary infiltration. Now, on the skin this condition, except in so far as it indicates the presence of a grave constitutional disease, is of no special importance. In the nostrils of a sucking infant, already debilitated and impoverished by the anæmia of syphilis, and depending upon its nutrition for the continuance of the miserable flickering life which was its original endowment, the same condition assumes the gravest significance.

The excessive supply of blood to the parts induces a catarrhal condition which shows itself in a thin, watery discharge, which, as the child during sucking is compelled to breathe through the nose, is rapidly dried into crusts. These become adherent, fill up and lessen the channel for the passage of air, and in so doing add to the rapidity and force of the respiration through the nose, and thus increase the tendency to the deposit of these crusts. The peculiar nasal, noisy respiration of the child has given the affection the popular name of snuffles. As the child can no longer breathe, or can breathe only with great difficulty, while sucking, it takes the breast only to drop it again immediately on account of impending suffocation.⁷⁵ As the disease progresses ulceration occurs beneath the crusts, and often involves the entire thickness of the delicate mucous and periosteal layers underlying the thin bones of the nose; perforation of these bones results, sometimes with caries to such an extent as to cause an entire loss of the nasal septum, with flattening of the nose—a symptom comparable to one which sometimes occurs in the tertiary period of adults, but produced, as we have seen, by other causes. In adults syphilitic caries and necrosis are usually due to lesions seated primarily in the osseous or subperiosteal tissues; in the child, at least in this instance, these tissues are involved secondarily.

⁷⁵ For an admirable description of the mechanism of this and other symptoms of coryza see Diday, op. cit., pp. 78-83.

Erythema, or roseola as it is differently called, is apt to present itself about the second or third week⁷⁶ after birth. As in the adult, it begins upon the abdomen in the form of little oval, circular, or irregular spots, dull red in color and disappearing upon pressure. Later the color becomes deeper, the eruption extends to the trunk and limbs, and, as exudation and cell-proliferation succeed to simple capillary stains, it ceases to disappear when pressed upon. It is often moist, owing to the thinness of the epidermis, sometimes excoriated. Occasionally it is confluent, and covers large areas with an almost unbroken sheet of deep-red color.

⁷⁶ Bassereau gives an instance of its occurrence within three days.

The diagnosis in the early stage is often difficult on account of the resemblance to the simple erythema of infancy. As the disease progresses, however, maculæ form here and there; the cell-infiltration involves the papillæ, several of which coalesce, forming flat papules; the nutrition of the superficial layers of the epiderm is interfered with, especially where it is thick, as on the palms and soles, and the eruption in those regions becomes scaly, and then the diagnosis is not difficult.

Papules and Mucous Patches.—In the ordinary evolution of the disease the next manifestation is usually the development of papules upon the general cutaneous surface and of mucous patches on the tongue, lips, and cheeks—probably also on other mucous membranes not exposed to examination. The papules are apt, for the reason already mentioned—the thinness and moisture of the skin—to be of the broad, flat kind, especially, as in the adult, in those regions where the elements of warmth and friction are superadded to the moisture, as in the folds of the skin about the genitalia, the neck, the flexures of the joints, etc. They are then moist, covered with a grayish secretion or a thin crust, and are in reality mucous patches. Occasionally they take on a little hypertrophy and develop condylomatous excrescences which closely resemble the simple acute condylomata of infants. In syphilis, however, the growth springs from a previously existing papule, which is not apt to be solitary, there being others in the neighborhood which will probably establish the diagnosis. The syphilitic condylomata also have a peculiar fetid discharge, resembling that of mucous patches and more or less characteristic.⁷⁷

⁷⁷ Van Harlingen, article "Syphilis" in the International Encyclopædia of Surgery, vol. ii. p. 560.

Mucous patches in the infant are among the most important of the early syphilitic lesions—not to the child itself, because they do not materially affect its health, save in those exceptional instances where they are accompanied by a marked degree of stomatitis, and thus interfere with its nursing. Their importance is due to the fact that they are almost constantly present, and they are thus by far the most frequent vehicle of contagion from the child to its nurse or to others with whom it may come in contact. At times they do not differ materially from the same lesion occurring in the adult, but lose much sooner their epithelial investment (on account of the delicacy and comparatively slight attachment of the epithelium at this stage), and they then appear as oval or irregular red, slightly depressed spots, distinct or coalescing, ulcerating or oftener covered by a false membrane. They especially affect the angles of the mouth and the sides and dorsum of the tongue; and indeed their disposition to select the former situation constitutes a diagnostic difference between them and non-specific stomatitis which is to be found in the sulci between the gums and cheeks and on the gums themselves—locations rarely invaded by mucous patches.⁷⁸ When the latter are ulcerating or are concealed by diphtheritic membrane, and are situated on the tongue, they may be mistaken for either simple or parasitic stomatitis. The diagnosis can often be made by the presence of other syphilitic symptoms—coryza, erythema, and especially papules. In their absence, however, it must be remembered that in simple stomatitis, the inflammation not being limited to special areas, the whole tongue is apt to be involved or a much larger portion of the buccal mucous membrane; and as there is no marked tendency to cell-proliferation in these cases, the accompanying exudation is apt to be serous or watery and to result in vesiculation—a condition never seen in syphilitic stomatitis. In the parasitic disease, too, the inflammation is less localized, there is more swelling and congestion, and the false membrane is said to be of a whiter color.

⁷⁸ Bumstead and Taylor, op. cit., p. 750.

No child that has even been suspected of having a taint of hereditary syphilis should be permitted to nurse at the breast of any one but the mother, to share its cup or nursing-bottle with other children, to receive the caresses of relatives or friends; and in this last restriction we would include the father, even if the suggestion⁷⁹ be true, that in the case of syphilitic children the protection from contagion probably extends to the male as well as the female parent. Paternity is sometimes a more doubtful problem than would seem probable, and even if the father were protected the husband might not be. The mucous patches, if any are found to exist, should be actively treated both locally and constitutionally, and during their demonstrable presence a most rigorous quarantine should be observed.

⁷⁹ Hyde, op. cit. See [p. 264].

Syphilitic condylomata are due to hypertrophic changes in the papules, which under the influence of heat and moisture in certain regions coalesce and become more elevated. They vary in size from an eighth of an inch to a quarter or even a half of an inch in diameter. Their surface is flat and covered by a crust or by an offensive secretion. They are found most commonly about the anus or at the angles of the mouth.

Pustular Syphilides.—A little later in the secondary period, usually at about the sixth week, but sometimes much earlier, the papules become transformed into pustules, the change taking place slowly, so that if examined at any time after it has begun the child will present an eruption which is markedly polymorphic, showing here and there yellowish or reddish-yellow maculæ left after the absorption of the cell-element of certain papules, at other places beefy-red papules at the height of their development, or papules crowned by a ring of desiccated and desquamating epidermic scales, and in still other regions pustules in various stages of formation. Or the various formative stages of the pustules may be passed through so quickly that the eruption will be almost entirely pustular, few if any unmodified papules being discovered. The pustules may remain distended with pus for a considerable time, after which they may wither and slowly disappear or may rupture and leave ulcerated surfaces. A number of these ulcers sometimes run together and make extensive patches covered with thick, dark-colored crusts. These patches may resemble areas of impetigo or of impetiginous eczema, but in those affections the crusts are usually thinner and of a lighter color, and the skin beneath them is generally on a level with the surrounding surface, bright red and glazed; while under the crusts of the syphilide will be found a more or less depressed or excavated ulcer, often covered with pus. The diagnosis may indeed often be made by gently detaching and raising one of the crusts and noting the character of the surface beneath. The erosion under the crusts of eczema heals over more readily and without leaving a cicatrix.

A so-called furuncular eruption⁸⁰ is said to appear at variable periods between the sixth month and the third year, but does not appear to me to be clearly differentiated from the large pustular syphilides with thickened and elevated bases on the one hand, or the ulcerating tubercular eruption on the other.⁸¹ They are all so rare in hereditary syphilis, at any rate, as to have little clinical importance.

⁸⁰ Bumstead and Taylor, op. cit., p. 750.

⁸¹ The distinction between the two forms is usually manifest if the development of the lesions has been observed; but even this fails in regard to the tubercular eruption. They both occur at the same period; they both begin similarly, the furuncles as "small nodules in the corium," the tubercles as "deeply-seated papules or nodules;" they both run on to ulceration and pursue a chronic course (Van Harlingen, op. cit., p. 561).

Iritis.—Another symptom of the secondary period, but of later development and of rarer occurrence than the syphilodermata which have been described, is iritis. In spite of its rarity this is extremely important, because it is frequently overlooked until it has reached such a stage that occlusion of the pupil results, and also because when it is recognized it constitutes an almost pathognomonic sign of syphilis.⁸² This statement may now be made unhesitatingly, although for many years it was contended that iritis, and even the still more characteristic symptom keratitis, were only associated with syphilis as coincidences, the constitutional disease, when hereditary, having no causative relation to the local condition.

⁸² "When primary iritis occurs in syphilis in young children it is almost always due to syphilis" (Soelberg Wells, Treatise on Diseases of the Eye, Philada., 1873, p. 173).

To Mr. Hutchinson belongs the credit of having first clearly developed the specific character of this trouble,⁸³ which, on account of the mildness of the attendant symptoms, is often overlooked. The sclerotic zone of congestion so marked in the adult, and therefore so valuable a diagnostic sign to the general practitioner, is very slight, sometimes absent; and as a consequence the attention of neither parent nor physician is attracted to the condition until in the more serious cases it has done irreparable mischief. In milder cases, particularly where the child is under mercurial treatment for concomitant symptoms of syphilis, it may run its course and escape notice altogether;⁸⁴ and it is possible that owing to this fact the rarity of the affection has been overestimated. It is also possible that in such cases changes occurring at this time may in some instances lay the foundation for some of the deeper-seated ocular troubles of later life.

⁸³ Med. Times and Gazette, 1860, July 14; Ophthalmic Hospital Reports, vol. i. pp. 191, 226; A Clinical Memoir on Certain Diseases of the Eye and Ear consequent on Inherited Syphilis, London, 1863. In the introduction to this volume Mr. Hutchinson states that acute iritis dependent on hereditary syphilis was first described in connection with its true cause by Mr. Lawrence, but, as from the date of that gentleman's first case (1830) up to 1863 but six cases had been recorded, the announcement had made but little impression on the profession.

⁸⁴ "In the cases of this form of iritis which are seen in ordinary eye-practice much damage has often been done by occlusion of the pupil and deeper mischief. Probably many of the slighter cases escape the notice of the parents and are not brought to the surgeon" (Mr. Edward Nettleship. See Hill and Cooper, p. 271).

If, however, attention has been attracted to the eyes, the diagnosis is not usually difficult. The pupil is irregular, especially under atropia; there are streaks of lymph, dulness, swelling, change of color, and on very careful inspection a faint pink zone may be seen in the sclerotic. The conjunctiva and cornea are generally clear.

Mr. Hutchinson's analysis of the twenty-three cases reported by him is still of interest as furnishing reliable data for prognosis. The average age at the time the iritis commenced was five months and a half. The oldest was sixteen months at the time of the outbreak, the youngest six weeks. In twelve cases but one eye suffered; in eleven both were affected. In seven cases (ten eyes) the cure was complete; in two or three other cases very slender adhesions remained; in twelve cases, in nearly all of which the patients came under care only at a late period of the disease, one pupil was permanently occluded by organized false membrane. In nearly all, coincident symptoms of syphilis of the skin or mucous membranes were present. Of the thirteen cases in which alone a history of the family is recorded, the affected infant was the only living child of his parents in twelve instances. In the only case in the whole series in which it is stated that there were other living children the mother had lost four infants out of seven live births.

The prognosis depends on the stage at which they come under treatment. The lymph if recent, no matter in what quantity, will probably be absorbed under mercurial treatment, which will often be of great benefit even in those cases in which a certain amount of organization has occurred.⁸⁵

⁸⁵ It will not be uninteresting, perhaps, to append the aphorisms regarding iritis in infants which Mr. Hutchinson at that time enunciated: 1. The subjects of infantile iritis are much more frequently of the female than the male sex. 2. The age of five months is the period of life at or about which syphilitic infants are most liable to suffer from iritis. 3. Syphilitic iritis in infants is often symmetrical, but quite as frequently not so. (In his article in Reynolds's System of Medicine, written in 1866, three years later, but revised in 1870, he describes it as "usually symmetrical," vol. i. p. 444.) 4. Iritis, as it occurs in infants, is seldom complicated, and is attended by but few of the more severe symptoms which characterize the disease in the adult. 5. Notwithstanding the absence of phenomena of acute inflammation, the effusion of lymph and the danger of occlusion of the pupil are usually very great. 6. Mercurial treatment is most signally efficacious in curing the disease, and, if recent, in procuring the complete absorption of the effused lymph. 7. Mercurial treatment previously adopted does not prevent the occurrence of this form of iritis. 8. The subjects of infantile iritis, though often puny and cachectic, are also often apparently in good condition. 9. Infants suffering from iritis should always show one or other of the well-recognized symptoms of hereditary taint. 10. Most of those who suffer from syphilitic iritis are infants born within a short period of the date of the primary disease in their parents.

We have now a group of symptoms characteristic of the secondary period of syphilis, or that extending from birth, or much more commonly from the age of three or four weeks to about the end of the first year. The syphilitic child during this time has several or all of the following symptoms: Coryza with snuffles; an erythematous, papular, or pustular eruption on the skin; mucous patches on the lips, tongue, cheeks, etc.; a marked tendency to general wasting; a hoarse cry or cough; senility of aspect; iritis. The majority of syphilitic children born alive die during this stage.

Before its termination, sometimes even at birth, other lesions have been noticed (especially those affecting the liver), which, however, may better be described in connection with the special organ or organs involved.

Succeeding this stage—i.e. beginning in about a year or eighteen months—comes an intermediate period, which extends to second dentition, to puberty, or even much later, and which is characterized rather negatively—that is, by the absence of symptoms—than otherwise. The evidence of the general diathesis will of course be present in the shape possibly of malnutrition, stunted growth, or retarded development, perhaps shown in the weazened or withered face, the sunken nose, the pallor of the skin, the premature loss of the upper incisor teeth or the malformation of the others if they have erupted.

There is but little tendency to recurrence or relapse of any of the secondary symptoms; and in certain cases, not a very small proportion, in which these symptoms have been light and have been well and thoroughly treated, this stage extends throughout life; or, in other words, as is frequently the case with the adult who has followed a proper course of treatment, the disease appears to terminate with the secondary stage. In other cases, however, it recurs, and the symptoms which it then presents may be taken up in connection with the different organs or tissues involved.

Syphilis of the ear is for obvious reasons not often discoverable until the patient has reached an age at which interference with the function of hearing becomes a noticeable phenomena. The only symptom likely to attract attention during the stage of inherited syphilis which we are now considering is a catarrh of the middle ear, which may have for its starting-point some inflammation, ulceration, or mucous patch of the pharynx, causing a temporary or permanent occlusion of the orifices of the Eustachian tubes.⁸⁶ This may lead to perforation of the membrana tympani, purulent infiltration of the mastoid cells, etc., and when accompanied by an otorrhoea which attracts attention to the ear will be easily discovered by the physician. These cases are, however, exceptional, otorrhoea only being present in nine out of Hutchinson and Jackson's⁸⁷ one hundred cases of inherited syphilis, and consequently but little is known about the frequency or gravity of lesions of the auditory apparatus in the secondary stage of this form of syphilis.⁸⁸ The changes which occur later on are chiefly those which involve either the nerves themselves or their distribution in the labyrinth.

⁸⁶ Bäumler, Ziemssen's Cyclopædia, vol. iii. p. 226.

⁸⁷ Hutchinson and Hughlings Jackson, Med. Times and Gaz., Nov. 23, 1861.

⁸⁸ Schwartze (quoted by Hill and Cooper) found also that otorrhoea was a rare complication in deafness from syphilis.

The affections of the middle ear and Eustachian tube are said to be contemporaneous with the keratitis which appears in the neighborhood of puberty,⁸⁹ while those of the nerve are somewhat later in point of time, and are almost always conjoined with retinitis, choroiditis, and optic neuritis. As usual when investigating or describing any subject relating to syphilis, Mr. Hutchinson's opinion and observation must be detailed. In 1863 he wrote⁹⁰ that it was only recently that he had thought of specially investigating the disorders of hearing in reference to hereditary taint, having had his attention called to a peculiar form of deafness, usually symmetrical, passing rapidly through its different stages and unaccompanied by any marked degree of pain or any external disease. He then reported eighteen cases of which he had notes. The oldest of these patients was twenty-seven, the youngest eight—the average time of development of the deafness from twelve to fifteen. Although the membrana tympani was in no instance quite normal, in none were there found adequate changes to account for the deafness. In all the Eustachian tubes were pervious. In nearly all the disease was symmetrical. This fact, together with the absence of discoverable lesions of the external or middle ear, seems to point conclusively to disease of the nerves themselves, or at least to a central cause.⁹¹ He adds: "With regard to the prognosis of heredito-syphilitic deafness, I believe that it is very unfavorable. When the disease was progressive I have rarely witnessed any permanent improvement or arrest. In most it has gone on to total loss of hearing, and this in several instances in spite of the cautious use of specific remedies almost from the beginning. From six months to a year would appear to be the usual time required for the completion of the process and the entire abolition of the function."⁹²

⁸⁹ Purves, Guy's Hospital Reports, 1875, p. 564; Pritchard, British Medical Journal, April 21, 1877.

⁹⁰ Clinical Memoirs on Certain Diseases of the Eye and Ear consequent on Inherited Syphilis, London, 1863, pp. 182, 183.

⁹¹ In the Lancet for Jan. 16, 1875, he reports a case of total deafness in a young woman of seventeen which had come on in ten months without pain or otorrhoea. He believes the disease of the organ of hearing to be parallel with those cases of choroiditis disseminata or of optic neuritis in which blindness is produced without pain or any external evidence of inflammation, and which are distinctly and positively associated with inherited syphilis.

⁹² Mr. Hinton, in his edition of Toynbee's work on Diseases of the Ear, states that at Guy's Hospital, of his aural patients, one in twenty is affected with deafness due to heredito-syphilis; that it usually makes its appearance between the tenth and sixteenth years; and that the great majority of the cases which he has seen have been females. He adds: "Patients suffering from this disease may, as a rule, at least when young, be at once distinguished by the amount of deafness which they exhibit. I know no other affection except fever which in a person under twenty brings on a deafness so rapidly and so nearly complete. In the course of a few weeks a girl previously hearing well will, without pain or known cause, become unable to distinguish words." In one of Dalby's cases total deafness came on in three weeks, previous to which hearing was normal. According to Pierce, the deafness is most apt to manifest itself between eleven and eighteen years of age. Troeltsch says that "l'audition du diapason par le vertex" is lost at an early date after the beginning of the disease, and that there are also often concomitant affections of the nose and pharynx.

Dalby⁹³ is said to regard syphilis as, next to scarlatina, the most fruitful cause of deaf-mutism as it occurs in children born with good hearing powers. "The patient usually becomes deaf in early childhood—after he begins to talk—or between this period and puberty."⁹⁴

⁹³ The Lancet, Jan. 22, 1876.

⁹⁴ Bumstead, op. cit., p. 734.

Syphilis of the Liver.—In 1852, Gubler published an account of the general appearances in syphilitic disease of the liver in new-born children, which was distinguished especially by increase in size and weight. This increase depended, as might be expected in this stage—that of general cell-proliferation—upon a proliferation of cells from the connective tissue between the acini, or from the adventitia of the interlobular vessels, this growth becoming transformed into connective tissue.⁹⁵ The change is quite analogous to what is taking place at the same time in the skin, the mucous membranes, and other tissues. Wilks has also described⁹⁶ a form of syphilitic disease of the liver which corresponds to that of Gubler, and in which the whole organ is infiltrated by a new fibrous tissue, producing a uniform and general hardening.

⁹⁵ Bäumler, op. cit., p. 186.

⁹⁶ Trans. Path. Soc., vol. xvii., 1866.

FIG. 8.

Section of an old gumma of the liver. a, a. Central caseous tissue of the gumma. v', v'. Its vessels. l. Boundary between the central portion and fibrous zone; this line of demarcation is marked in places by an opening or cleft. t, t. Connective tissue of the fibrous zone which entirely surrounds the central part. v, v. Small vessels of this zone. c. An arteriole of the fibrous zone. f, f. Quite large biliary vessels included in the fibrous zone. t'. Fasciculi of connective-tissue fibres running parallel with the surface of the caseous part. At b and d the fasciculi of fibres of the fibrous zone penetrate into the central caseous part. e, e. Tissue of hepatic cells interrupted by bands of fibrous tissue, m, m. X 12.

As described by Gubler,⁹⁷ the liver in such children is hypertrophied; hard, resistant to pressure, so that it cannot be indented; elastic, so that it rebounds; creaks, but does not bleed, when it is cut into, and presents the yellow color and the semi-transparence of flint. There are seen on a yellowish ground a number of small white granulations like grains of wheat, which a histological examination shows to be formed by an accumulation of embryonic cells in the spaces which separate the hepatic acini. Injections reveal the fact that the vascular network has become almost impenetrable, the capillaries obliterated, the larger vessels diminished in calibre. Fibro-plastic matter is found throughout the organ in large quantity. In consequence of these conditions—the compression of the hepatic cells and the destruction of the vessels—the secretion of bile is stopped, and the gall-bladder is found after death to contain a pale-yellow liquid consisting of bile mixed with an excess of mucus. This form of hepatitis has thus far been observed almost exclusively in infants. Cornil says⁹⁸ that he has had frequent occasion to examine such cases of hepatic syphilis, and describes them as follows: "The hepatic acini, in the normal state, are in contact except at the prismatic spaces which are formed by their union—spaces in which the capsule of Glisson forms an envelope to the afferent portal vessels of the lobuli. It is in these spaces that the round lymph-cells form and collect into small nodules representing microscopic gummata. The cells at the centre of the new formation are sometimes granular. This neoplasm is seated about the ramifications of the portal veins, which in consequence also present thickened walls with newly-formed cells in their external tissues. The small granules above mentioned are not always visible to the naked eye, and in their places are only seen, about the perilobular capillaries of the portal vein, an excessive number of embryonic cells." In addition to this interstitial sclerosis or interstitial infiltrating hepatitis there is an inflammation of the liver depending upon the presence of gummata—gummous hepatitis—which occurs in two forms: one in which very small and very numerous nodules are present, situated along the course of the fibrous seams, the prolongation of the capsule, and another in which there are two or three large circumscribed tumors. This form of hepatitis is always accompanied by the interstitial form, although the latter may be only slightly developed.⁹⁹ The gummata, though not infrequently found in the liver of new-born children, are more likely to develop later, at from about the eighth to the twelfth year.

⁹⁷ Mémoires sur une nouvelle Affection de Foie, and Gaz. Méd. de Paris, 1852.

⁹⁸ Op. cit., Am. ed., p. 370.

⁹⁹ It does not differ essentially, either pathologically or clinically, from the same lesion in adults.

Rochebonne¹⁰⁰ describes the following symptoms of syphilitic hepatitis in infants: A deep wine-colored venous stain and oedema of the lower extremities, often accompanied by pemphigus; ascites due to mechanical obstruction of the circulation, as in cirrhosis; a more or less pronounced chloro-anæmic appearance of the face; and the presence in the urine of albumen and hæmato-globulin. Vomiting may occur, and constipation alternating with diarrhoea has been observed. Icterus, symptomatic of the affection, has not been observed.

¹⁰⁰ Quoted by Bumstead and Taylor, p. 758.

Bäumler says:¹⁰¹ Implication of the peritoneal coating of the liver may be recognized by the pain in the hepatic region. In new-born children—unless, possibly, there may be some enlargement of the liver—the only local symptoms, often, are those due to peritonitis—screaming, drawing up of the legs, vomiting. In those cases it is not rare for the peritonitis to become diffuse.¹⁰²

¹⁰¹ Op. cit., p. 194.

¹⁰² In an article on "Inherited Syphilis" in the British and For. Medico-Chirurgical Review, 1875, p. 28, it is said: "Of the liver the lesion consists in enlargement and induration of the organ in whole or in part, due to the development of fibro-plastic material between the cells of the acini, with obliteration of the vessels and interference with the secretion of bile. This condition is generally doubtful during uterine life, and is rapidly fatal. The symptoms are vomiting, diarrhoea, and tympanitis, but, strange to say, no jaundice. The enlarged and indurated organ may be felt by palpation. It is probably in this connection that the peritonitis described by Simpson as occurring in inherited syphilis is found."

Hill says:¹⁰³ "The symptoms are mainly those of functional derangement of the organ, with alteration of its bulk."

¹⁰³ Op. cit., p. 163.

Hutchinson¹⁰⁴ has described cases in which in young persons the subjects of hereditary syphilis there has been great hepatic enlargement which has subsequently wholly disappeared. He finds it difficult to believe that there is any kind of gummous growth in such cases, and feels obliged rather to fall back upon the hypothesis of mere vascular turgescence. In one such case the liver occasionally was so large as to be visible as the patient lay on his back in bed.¹⁰⁵

¹⁰⁴ Path. Transactions, 1877, p. 309.

¹⁰⁵ Illustrative cases of this condition may be found in the Med. Times and Gazette, Dec. 22, 1877.

It seems much more likely that the enlargement is due to an exceptionally active cell-proliferation, which does not, however, go on to organization, but may be just as susceptible of absorption and resolution as are the papules or maculæ of the skin. A portion of the enlargement may be due to a passive congestion caused by the presence of this cell-accumulation.¹⁰⁶

¹⁰⁶ Barlow (Path. Trans., 1877, p. 355) has suggested that the engorgement is only a preliminary stage of the fibrous thickening, and may disappear either with or without leaving permanent contractions or adhesions in its wake.

As to the diagnosis of hepatic syphilis in infants, I am disposed to agree with Cornil, who says:¹⁰⁷ "The symptoms are null, or they are identical with those of local and general troubles so often observed in children who have poor or insufficient nourishment. The only physical sign which properly belongs to hepatic syphilis is, when it exists at all, increase in the size of the liver."

¹⁰⁷ Op. cit.

Syphilis of the Bones.—Until the publication in 1870 of the researches of S. Wegner,¹⁰⁸ an assistant of Prof. Virchow, diseases of the osseous system due to hereditary syphilis were either ignored or denied by the various writers upon this subject.¹⁰⁹ Valleix, Bargione, Ranvier, and Guéniot had indeed recorded cases of bone disease occurring at the points of junction between the epiphyses and diaphyses and in the costal cartilages, but it remained for Wegner first fully to describe the pathological changes which occurred there, and to differentiate them from those due to rickets or scrofula. His memoirs recognized three stages of alteration in the long bones:¹¹⁰ 1st. While in the normal state the boundary of the hyaline cartilage is distinctly marked by a line which indicates the direct transformation of the cartilaginous tissue into a spongy tissue, the unaided eye being unable to distinguish a spongio-calcareous layer, in new-born syphilitic children, on the contrary, the bones are seen to have a spongio-calcareous layer interposed between the bone and cartilage, measuring two millimeters in thickness. This is a zone of calcifying cartilaginous material more extensive than in the normal state. 2d. These same changes become more distinct and more extensive. The unnaturally thick layer of calcareous material continues to grow. There is proliferation of the cartilaginous trabeculæ, abundant calcification of the cartilage, too early and irregular ossification of the intercellular substance of the cartilage, and at the same time an arrest of the normal formation of bone which should be going on from the epiphysial cartilage. 3d. There is now added, by extension of these processes, a thickening of the perichondrium and periosteum at the extremities of the long bones and at the junction of the ribs with the costal cartilages. In consequence of the interference with nutrition occasioned by these changes atrophy and fatty degeneration of the cartilage-cells occur, and they form between the epiphysis and diaphysis a necrosed mass which irritates the living bone. This causes osteo-myelitis, which frequently results in a separation of the epiphyses. Occasionally pus is produced in such quantity as to perforate the periosteum, escape into the surrounding tissues, and become superficial. He terms the entire process an osteo-chondritis.

¹⁰⁸ Virchow's Archiv, 1870, B. 50, S. 305: "Ueber hereditäre knochen Syphilis bei jungen Kindern."

¹⁰⁹ Diday says: "Affections of the bones are so rare in children with inherited syphilis that the annals of medicine scarcely offer five or six well-authenticated cases of caries or periostitis" (op. cit., p. 83). Referring to this statement, Mr. Hutchinson remarks: "So different has been my own experience from this that I may say that we are scarcely ever without a severe example of it in the wards of the London Hospital" (Illustrations of Clinical Surgery, London, 1875, p. 47).

¹¹⁰ Cornil, op. cit., p. 282 et seq.

Waldeyer and Köhner,¹¹¹ after examining twelve cases, confirm in the main these investigations of Wegner, but interpret the changes as arising rather from the formation of a gummous tissue between the epiphysis and diaphysis than from an osteo-chondritis. The tissue-death which occurs later, the atrophy of the cells, etc., they compare with the same modifications observed in syphilomata.

¹¹¹ "Beiträge zur Kenntwiss der hereditäre knochen Syphilis," Virchow's Archiv, B. 55, S. 367.

Parrot¹¹² in a number of exceedingly valuable papers has repeated and greatly extended these observations. He places especial importance upon the formation of osteophytes, which, he says, in the first stage envelop the diaphyses of the long bones, especially at their inferior extremities. In the succeeding stage the new bony layers are more porous; a gelatinous degeneration affects the epiphysial cartilage and the spongy bones at a point where they are in contact; the epiphyses tend to separate from the diaphyses. This solution of continuity results in a characteristic pseudo-paralysis, with curvatures, abnormal twistings, and preternatural mobility of the bones, with loss of the power of locomotion. Then the osteophytes increase in size by the formation of several layers, thus enlarging the inferior extremities of the long bones. He describes the general process as consisting, first, of a periosteo-genesis—a formation of osseous tissue from the periosteum; next of a chondro-calcosis—a calcareous incrustation of cartilage; and finally of a gelatiniform degeneration and softening of the bone, with diaphyso-epiphysial disjunction.¹¹³

¹¹² Société de Biologie, June 1, 1872; Société anatomique, 1873, p. 92; Archives de Physiologie, 1876, vol. iii. pp. 138, 139; Revue mensuelle de Médecine et de Chirurgie, 1877; Pathological Trans., 1871, vol. xxx. p. 339, etc., etc.

¹¹³ Cornil (op. cit.) coincides in the main with this description.

Taylor¹¹⁴ sums up the results of his observations as follows: "In the first stage we have a simple hyperplasia of cells with irregular deposition of lime salts; in the second, an intensification of this condition; and in the third, a new element—namely, the abnormal proliferation of all the elements of the tissues, with an infiltration of granulation-tissue into the medullary spaces following the vessels."¹¹⁵

¹¹⁴ Syphilitic Lesions of the Osseous System in Infants and Young Children, New York, 1875, p. 134.

¹¹⁵ Verraguth (Archiv für Path. Anat.) describes the first step as an excessive formation of vessels in the cartilage and a corresponding overgrowth of the cellular elements. This becomes inflammatory, and constitutes a primary syphilitic chondritis, the changes in the medulla of the bone being degenerative and secondary to the affection of the cartilage. Still other observers have described the process, each with minor modifications; but as they are of no clinical importance, it does not seem worth while to quote them.

We see, then, that, setting aside minor points of difference, these observers all coincide in describing this condition as one essentially of the nature of syphilitic bone troubles with which we are familiar in the acquired form of the disease, consisting primarily and throughout of an unnatural accumulation of cell-elements, which in the later stages by their pressure produce various degenerations of surrounding structures, and which, as they occur during the process of bone-formation, are accompanied by irregular and abnormal deposition of lime salts. They especially affect the regions mentioned—the junctions of the epiphyses and diaphyses—because at that time those points are the seat of great physiological activity. Syphilis, indeed, throughout its entire course is notably subject to similar influences, as one example of which I may instance the preference displayed by the periostitis which results in nodes or in caries for the subcutaneous bones, the tibia, clavicle, cranium, etc.; or, in other words, for those which are subject to frequent traumatisms—trifling, perhaps, but sufficient to determine a slight hyperæmia, which is followed by abnormal cell-proliferation or accumulation.

The symptoms which obtain in this condition of syphilitic osteo-chondritis are as follows: The child may be attacked during intra-uterine life, and in that event the osseous lesions will probably be coincident with other syphilomata and with placental disease of sufficient gravity to destroy life.¹¹⁶ If the child is born alive, the first development of the disease will probably be noticed as a swelling at the diaphyso-epiphysial junction of one of the long bones, which in the emaciated subjects of hereditary syphilis is often visible, and can always be discovered by palpation. The bones most frequently attacked are the humerus, radius and ulna, tibia and femur, but the clavicle, ribs, sternum, and bones of the metatarsus and metacarpus are also often involved, and much more rarely the frontal and parietal. The more pronounced the syphilis of the parents, or the nearer the date of conception to the time at which their infection occurred, the more probable is it that several bones will be affected, and the more unfavorable the prognosis as respects the life of the child. Indeed, it has been noticed that "in stillborn infants and in those dying soon after birth the majority, or even all, of the long bones are affected."¹¹⁷

¹¹⁶ Pollnow found osteo-chondritis in 35 out of 50 syphilitic foetuses (Der Hydrops Sanguinolentes foetus, Berlin, 1874, quoted by Hill and Cooper, op. cit., p. 352).

¹¹⁷ Bumstead and Taylor, op. cit., p. 767.

The swelling is found to consist of a ring or collar which more or less completely surrounds the bone, is apt to be smooth rather than irregular, and when two bones situated near to each other are simultaneously affected may conjoin them. This condition persists during the first stage of pathologists, and passes with greater or less rapidity into the second stage, in which the swelling, the cell-proliferation, reaches its height. This may take, in cases uninfluenced by treatment, several weeks or even months. Under the use of mercurials and iodide of potassium they usually subside rapidly. During this second stage, however, owing to the proximity of the swellings to the joints, a moderate amount of synovitis is often present. This affects chiefly the elbow and the knee, but may appear in any joint. It is also readily influenced by specific treatment and well-regulated pressure.

When the third stage is reached, or that of the formation of granulation-tissue, with degenerative changes of the cartilages and of the bones themselves, deformity often becomes more marked. There are unnatural curves or angles in the bones, with more or less complete separation at the point of junction. Where many bones are affected in this way, the resulting deformity is extreme and the patient may be absolutely powerless, a condition of pseudo-paralysis supervening in which the limbs lie motionless or swing about like the arms or legs of a doll when the child is carried.

When the swelling does not undergo absorption, the superjacent tissues sometimes become involved, abscesses form and make their appearance externally, extensive necrosis of the shaft of the affected bone takes place, and the little patient usually dies of hectic, pyæmia, or exhaustion. When the cranial bones are involved, the disease is apt to limit itself chiefly to the stage of osteophytic formation, the immovability of the bones probably favoring the organization of the new cell-growth rather than the production in it of inflammatory changes. The growths are met with chiefly in older children than those affected with the form of osteo-chondritis just described; they affect the periphery of the liver, and are found most usually around the anterior fontanel, and later on the parietal and frontal eminences. The sutures are sometimes completely soldered together.¹¹⁸ The osteophytes vary in thickness from a quarter of an inch to an inch, or are even larger.¹¹⁹

¹¹⁸ In a case reported by Barlow it was not possible at the autopsy to discover the point of union (Path. Transactions, 1879, p. 339).

¹¹⁹ These conditions may all result in a child the subject of acquired syphilis, but are apt to be milder, to involve fewer bones, and to yield more readily to treatment. This would of course be expected, inasmuch as the same difference in favor of the acquired form, as compared with that which is inherited, extends to all the lesions. As Diday succinctly expresses it: "In the one case the poison vitiates only the elements of nutrition; in the other it vitiates at the same time those of formation and those of nutrition." It would exceed the limits of the present article to describe acquired syphilis in children.

The most important differential diagnosis to be made in these cases is between the rachitis of young children and the form of syphilis in question. Much difference of opinion still exists as to the relation between these diseases, syphilis being claimed, on the one hand, as having in the majority of cases a definite causative influence, while, on the other, the existence of this relation is denied. When we come to contrast the pathology of the two diseases, we can readily understand why they should be confounded, the minuter changes which occur being essentially the same—viz. cell-proliferation and accumulation, with subsequent inflammatory changes, associated with irregular deposits of lime salts.

Compare, for example, the description of the pathology of bone diseases in inherited syphilis already given (pp. [287], [288]) with the following terse summary of the changes which take place in rickets in cases where no suspicion of syphilis exists, either ancestral or acquired: "The changes are more distinctly noticed at the epiphyses than in the diaphyses. Instead of the regular stages and distinct boundaries observed in the normal development of bone, there is a singular disorderly commingling of the exaggerated cartilage-proliferation and transition substance, with calcification. The cartilage-cells, stimulated to excessive multiplication, are transformed, some into bone-corpuscles, some into medullary cells, and others into connective-tissue forms. The same process is in active operation in the deep periosteal layers, the material accumulating to such a degree as to add much to the thickness of the shaft."¹²⁰

¹²⁰ Agnew's Surgery, vol. i. p. 1030.

The points of resemblance are manifest, just as they are between a syphilitic and a variolous pustule, but they end in both cases when we come to study the evolution of the phenomena either from an anatomical or from a clinical standpoint. They may be expressed as follows in tabular form:

OSSEOUS LESIONS DUE TO INHERITED SYPHILIS.	RICKETS.
The swellings, particularly those of the long bones, show themselves at or soon after birth.	Rarely appear before six months, generally still later.
A history of syphilis or evidence of existing syphilis in one or both parents.	No such history necessarily.
Preceded or accompanied by snuffles, coryza, and cutaneous and mucous lesions.	No such prodromata.
No such prodromata in most cases.	Pallor, restlessness, sweating, nausea, diarrhoea, etc. constitute a combination of symptoms which often precede the bone disease.
Cachexia absent or moderate.	Cachexia marked.
Physiognomical peculiarities of syphilis present.121	Not present as a group.
Circumscribed tumors on frontal and parietal bones, rarely on occiput.	Cranial bones thickened in spots, usually upon the occiput.
Ribs not markedly affected.	All or nearly all involved.
Swellings on long bones or extremities irregular.	Extremities symmetrically enlarged.
Disease of ribs, when existent, not ordinarily coincident with that of other bones.	Nearly always so.
Fontanels close at usual period.	Closure delayed.
Other syphilitic symptoms present; enlargement of phalanges, metatarsal bones, etc.	Syphilitic symptoms absent.
Often accompanied by sinuses, synovitis, abscesses, cutaneous ulcers, etc.	Little external or surrounding involvement.
Generally disappears by resolution, without leaving any permanent change.	Usually leaves some bending of shaft and distortion of the neighboring joint.
Mortality among children in whom many bones are involved is very great.	Much less.
Specific treatment useful.	Of no benefit.
In the first stage there is an exuberant calcification of the ossifying cartilage, causing necrosis of the new-formed tissue and a consecutive inflammation, which terminates in the separation of the epiphyses.122	This is less marked. There is formed, instead, a soft and non-calcified osteoid tissue.

¹²¹ See p. [313].

¹²² This table is founded on one which I added to the translation of Cornil made by Dr. Simes and myself, and is compiled chiefly from the excellent work of Dr. Taylor already alluded to.

The diagnosis of the bone lesions of hereditary exostosis can readily be recognized in a short time by noting the fact that they are stationary, even if their later appearance, larger size, the absence of syphilitic history or symptoms, and the resistance to specific treatment left us in doubt.

The diagnosis from accidental separation of the epiphysis, or from fractures, may be made from the history of the case.

In cases of separation of the epiphysis, complicated with suppuration, sinuses, etc., the trouble may be mistaken for a similar condition due to non-specific inflammation. In all the recorded instances, however, the latter has occurred much later in life, is attended with much more acute inflammatory symptoms, lymphangitis, etc., and is of course without concomitant symptoms of syphilis. In both these cases there is a decided osteo-periostitis, and as so much depends on the early and vigorous use of specific treatment, it may be worth while to contrast the two forms of the disease.

SYPHILITIC OSTEO-PERIOSTITIS.	NON-SPECIFIC OSTEO-PERIOSTITIS.
Occurs in infants under three months of age.	No instance of its occurrence in children under one year of age.
History of syphilis in child and its parents.	No history of syphilis; sometimes a history of traumatism.
Implication of other bones.	Usually confined to one bone.
Coincident with the development of the shaft of the bone.	Coexists with the ossification of the epiphyses.
Other lesions of syphilis: nodes, skin eruptions, etc.	No such symptoms.
All the local symptoms comparatively mild.	Pain, redness, and swelling very marked.
Disease sharply localized.	Involves neighboring parts.
Lymphatics of limb unaffected.	Lymphangitis present.
Beneficial effect of specific treatment if employed early.123	No such effect.

¹²³ Cornil, op. cit., p. 274.

Syphilitic dactylitis in the inherited variety of the disease, as in the acquired, consists of two varieties. The one of these which usually appears earlier involves chiefly the periosteum and the fibrous and integumentary structures surrounding a joint, usually a metacarpo- or metatarso-phalangeal articulation, involving a phalanx, and is characterized by slow, almost painless, swelling and discoloration of the affected member. (Fig. 9.) This is due to a gummous infiltration which, after absorption under proper treatment, leaves the toe or finger temporarily stiff, but not permanently disabled.

The second form is a specific osteo-myelitis, with periostitis, coming on later, and often destroying the bone or the articulation involved. (Fig. 10.)

FIG. 9.

FIG. 10.

From Bumstead on Venereal Diseases, illustrating Syphilitic Dactylitis.

The absence of acute inflammatory symptoms in the first variety distinguishes it from paronychia, whitlow, and gout. Rheumatoid arthritis begins in the joints, is associated with other symptoms; deformity of the fingers comes early in the disease, and there is a teno-synovitis with contraction.

The second variety might be taken for enchondroma or exostosis, but these growths increase much more slowly, involve only a limited portion of the bone, are of greater density, and are much more strictly circumscribed.

As a rule, especially in cases which are recognized early and treated actively, the prognosis is good. Iodide of potassium should be used in combination with mercury.

Syphilis of the Teeth.—Syphilis of the teeth has its chief interest to the general practitioner from its very important bearing on diagnosis. As manifesting itself at an age when the child is not apt to present the active and unmistakable cutaneous and mucous lesions of the disease, and when, consequently, its recognition is often extremely difficult, this diagnostic importance is greatly increased.

The teeth of the first dentition, although exhibiting the usual signs of interference with nutrition in their irregular development, opaque and chalky enamel deficient in quantity and unevenly distributed, soft and friable dentine, incongruity of size individually and relatively, and proneness to decay, do not often display any distinctive evidence of syphilis. The same conditions may, and often do, depend on other causes, and are commonly associated with various cachexiæ—the strumous, gouty, rheumatic, rachitic, etc.—and even with other slighter ailments tending to produce imperfect assimilation and malnutrition.

In the permanent teeth, likewise, the same condition may be due to the same causes. Stomatitis, however produced—by mercury, by gastro-intestinal derangements, by local irritation of any kind—is apt to result in imperfectly organized dental structures. Mercurial teeth, for example, are usually irregularly aligned, horizontally seamed, honeycombed, craggy, malformed, of an unhealthy dirty yellow color, separated too widely, and deficient in enamel.¹²⁴ The diseases of childhood, especially the eruptive fevers, eclampsia, typhoid fever, etc., by temporarily arresting or greatly interfering with nutrition during the developmental period of the teeth, often cause horizontal furrows across their crowns, which are, of course, persistent throughout life, and mark indelibly the influence of such disorders on all the formative processes.

¹²⁴ The latter defect is particularly noticeable on the cusps of the sixth-year molars. (See note on "Syphilis of the Teeth," by Dr. James W. White, in Am. ed. of Cornil, pp. 287-290.) The discussion as to the effect of mercury in producing the condition of the teeth known as honeycombed is still going on, but the evidence seems to point clearly to a direct connection with the administration of mercury in infancy, either for syphilis or in excessive doses as a purge, or in some of the teething powders, which often consist of calomel and opium. Mr. Hutchinson, at a meeting of the Odontological Society (see Proceedings for 1877, p. 249), gave an interesting description of the way in which the supposition was arrived at. Lamellar cataract is a disease which affects the eyes of children who have suffered from convulsions: it was noticed that in cases of that form of catarrh there were also honeycombed teeth, and it was thought that the convulsions, the cataract, and the honeycombed teeth were all due to the same unknown cause. At last a few exceptions were found—patients with cataract, but with good teeth, and then some who had had convulsions only, and yet had honeycombed teeth; lastly, it was noticed that most of the patients had been treated with mercury. So it came to be recognized that the honeycombed teeth were only accidentally associated with the cataract, and that they were, in fact, the result of the mercury which had been given to cure the convulsions. The same gentleman figures a case of this disease in his Illustrations of Clinical Surgery (London, 1875), and thus describes it (p. 55): "The present state of his permanent teeth is so characteristic as to deserve more detailed description. The change about to be mentioned affects all the incisors, canines, and first molars of both upper and lower jaws, the bicuspid being scarcely implicated at all; the second molars are also quite healthy. In the first molars the alterations consist of deficiency of enamel on the upper surface of the crown and the presence of spines of uncovered dentine. In the case of the incisors a considerable portion of the crown of each tooth is totally devoid of enamel, and its dentine is also deficient to some extent, so that the teeth are thin, sharp-edged, and of a dirty yellowish color. The transition from the enamel-covered to the diseased part occurs suddenly in a horizontal line at some little distance from the crown of each tooth; the position of this line or step being in each tooth nearly at the same distance from the gum. The general effect when all the teeth are seen together is as if a string had been tied around them when soft and the distal part had withered."

None of these conditions, however, are in the least degree characteristic of syphilis, the special expression of which in the mouth is to be found only in the permanent upper median incisors. For the recognition and description of the peculiarities of these teeth in the subjects of inherited syphilis we are indebted, as we are for so much else of inestimable value in the study of the disease, to Mr. Hutchinson. In 1863, in a memoir on Syphilitic Diseases of the Eye and Ear, he wrote as follows¹²⁵ concerning the symptoms which, in a suspected case, would aid in determining the diagnosis: "By far the most reliable amongst the objective symptoms is the state of the permanent teeth if the patient be of age to show them. Although the temporary teeth often, indeed usually, present some peculiarities in syphilitic children of which a trained observer may avail himself, yet they show nothing which is pathognomonic, and nothing which I dare describe as worthy of general reliance.¹²⁶ The central upper incisors of the second set are the test teeth, and the surgeon not thoroughly conversant with the various and very common forms of dental malformation will avoid much risk of error if he restricts his attention to this pair. In syphilitic patients these teeth are usually short and narrow, with a broad vertical notch in their edges and their corners rounded off. Horizontal notches or furrows are often seen, but they, as a rule, have nothing to do with syphilis. If the question be put, Are teeth of the type described pathognomonic of syphilis? I answer unreservedly that when well characterized I believe they are. I have met with many cases in which the type in question was so slightly marked that it served only to suggest suspicion, and by no means to remove doubt; but I have never seen it well characterized without having reason to believe that the inference to which it pointed was well founded."

¹²⁵ Chapter on "The Means of Recognition of the Subjects of Hereditary Syphilis during the Tertiary Stage," p. 204. Before this, however, he had called attention to the same peculiarities in a paper on "The Means of Recognizing the Subjects of Inherited Syphilis in Adult Life," Medical Times and Gazette, London, Sept. 11, 1858, p. 265.

¹²⁶ So far as I know, the only recorded instances by reliable observers of the temporary teeth presenting the peculiar characteristics of syphilis are as follows: In the Transactions of the Odontological Society of Great Britain, vol. ix., 1877, p. 258, Mr. Oakley Coles described a case—without, unfortunately, giving details—in which the "peg-shaped temporary teeth were very characteristic of syphilis." Mr. Coles's abilities are well known, and the fact that at the previous meeting the society had been addressed by Mr. Hutchinson on this very subject would seem to indicate that he was quite familiar with the importance of his statement.

M. Fournier calls attention (Archives de Derm. et Syph., Sept. 25, 1883) to a cast (No. 48) to be found in the collection of M. Parrot in the museum of the Foundling Hospital at Paris. This displays the dentition of a child twenty-seven months old, in which the two upper median incisors are typical Hutchinson teeth. M. Fournier adds that later researches show unmistakably that the temporary teeth may be affected by hereditary syphilis in the same manner as those of the second dentition, although not more than one case of the former is recognized to fifteen or twenty of the latter. He thinks, however, that this proportion would be greatly modified if in the autopsies of young children the alveoli were opened to examine the embryonic teeth. M. Parrot by this means claims to have often discovered lesions of the milk teeth.

As a matter of course, so positive a statement in a matter of such gravity and importance excited considerable criticism, and the views of Mr. Hutchinson have never been without earnest and often able opponents; but it is safe to say that time has only served to place them on a surer foundation and to enhance their value in the eyes of the profession. That they have undergone no material change in the mind of their distinguished author is shown by his expressions of opinion during the debate on syphilis in the London Pathological Society in 1876,¹²⁷ and still later by the following memoranda which he gives as a guide in diagnosis:¹²⁸

"1. No special peculiarities are to be looked for in the first set of teeth.

"2. There can be no more serious blunder than to imagine that bad teeth in proportion to their badness of form are to be suspected of syphilis.

"3. The upper central incisors are the only teeth which are positively characteristic. The others may afford corroborative testimony, but are not to be relied upon alone.

"4. The chief peculiarity is a general dwarfing of the tooth, which is both too short and too narrow, and, from its sides slanting together, presents a tendency to become pointed. This tendency to pointing is always defeated by the cutting off of the end, the truncation being usually effected in a line curved upward, so as to produce a single shallow notch. At the bottom of this notch the enamel is deficient and the dentine exposed, but there is no irregular pitting, as in stomatitis teeth.

"5. The malformations are unusually symmetrical and affect pairs of teeth. The two central incisors resemble each other, and the two laterals are also alike. If any defect passes horizontally across all the incisors at the same level, and affects them all alike, it is probably not due to syphilis.

"6. In syphilis the lateral incisors usually show little or no malformation.

"7. The occurrence of the peculiarities due to syphilis and those due to mercury in the same mouth are exceedingly common."

¹²⁷ London Lancet, 1876, pp. 56 and 535.

¹²⁸ Illustrations of Clinical Surgery, fasciculus xi., London, 1878.

The great importance of the subject seems to me to justify one more quotation, as showing the opinion at a very recent date of men well qualified to judge of the correctness or inaccuracy of these statements. Mr. C. Macnamara and Dr. Thomas Barlow¹²⁹ say: "The characters of the teeth are so valuable when present that it is important to have them clearly noted—the more so that, in spite of Mr. Hutchinson's clear description, they have been much misrepresented. It may be pointed out—(1) That only the upper median permanent incisors are characteristic, and sometimes only one of them is typical, of the disease; (2) that these teeth are generally a little apart, instead of being in apposition, and are more or less dwarfed; (3) that in a typical specimen the width of the cutting edge is narrower than the width of the tooth as it emerges from the gum; (4) that a typical syphilitic tooth presents a single notch, not a serrated margin; and that occasionally, if the notch has not been actually scooped out, there is a little lunula-shaped area which may readily become a notch; (5) finally, that although such teeth, when present, are absolutely pathognomonic, the existence of normal permanent upper median incisors by no means excludes the existence of hereditary syphilis."

¹²⁹ On behalf of the Collective Investigation Committee, who have issued a circular designed to elicit information as to the effect of syphilis on the civil population of Great Britain. This circular has been sent to physicians, and contains queries as to various points relating to the symptoms of inherited syphilis; among others as to the existence in any given case of "notched, dwarfed upper median incisors," which, with or without other symptoms, would establish the diagnosis of that case. The observations above quoted are explanatory of this question (The British Medical Journal, Dec. 16, 1882).

I believe this may fairly be taken to represent the general belief at the present day among those best qualified to pronounce upon the merits of the case; and I may say that it is unqualifiedly my own opinion, arrived at after some experience and considerable investigation into the literature of the subject. It is not uncommon, however, to hear doubts expressed as to the value of this sign in the diagnosis of syphilis, and at intervals articles are written or papers read to prove that it is not of the uniform and distinctively conclusive significance that has been attributed to it.¹³⁰

¹³⁰ "I cannot say more in favor of the diagnostic values of these teeth than that, when present in typical form, they have a certain weight in favor of the existence of hereditary syphilis in the given subject" (Van Harlingen, article "Syphilis" in Internat. Encyc. of Surgery, vol. ii. p. 565). "It has been the custom from time to time since Mr. Hutchinson made his observation to question the validity of his views, both as to the fact of interstitial keratitis being due to hereditary syphilis, and as to the diagnostic values of the so-called characteristic teeth. Thus, it has been asserted, not only in England, but on the Continent, and especially in Germany, that the disease may be the result of malnutrition in scrofulous and rickety subjects; and it has been maintained that the malformation of the teeth is the simple arrest of development in a perverted constitution from other causes than syphilis" (Bumstead and Taylor, op. cit., p. 701). Garretson says (Oral Surgery, p. 316): "Observations will be found to greatly vary concerning the existence of any constancy in phenomenal expressions of the teeth in this relation."

M. Magitot, who has for some years been supposed to pay especial attention to this subject, has recently written an elaborate paper (Gazette des Hôpitaux, Sept. 29, Oct. 4, 11, and 18, 1881) to prove (1st) that dental erosions, as he calls them, are not due to syphilis; and (2d) that they are due to, or are almost invariably associated with, infantile convulsions. He has collected a number of interesting facts, but a very careful study of his article and inspection of his diagrams have convinced me that he does not recognize at all the special peculiarities of the Hutchinson teeth, but includes under his title of "erosion" a variety of widely-differing conditions. He has altogether misinterpreted Mr. Hutchinson's views as stated in his Memoirs on Certain Diseases of the Eye and Ear due to Inherited Syphilis, and represents him as at that time (1863) believing that the cause of the change in the incisors was a mercurial stomatitis. The quotation on p. [294] sufficiently refutes this absurdity. Of course the paper as an argument against the syphilitic origin of these teeth is without the slightest value.

Corroborative evidence—which, to my mind, is very strong as coming from men whose opportunities for observation of dental peculiarities are almost unlimited—is found in the writings of those gentlemen who have devoted themselves to dental and oral surgery exclusively. Mr. Henry Moon¹³¹ says:¹³² "My observations on this subject extend over some twelve years, and include some hundreds of cases; and although, in some details as to the manner of causation, I may differ from the view published by Mr. Hutchinson (before knowledge on tooth-development was advanced as it is at present), yet I must coincide entirely with his general conclusions." "The question really is this: Is there one peculiar conformation of the teeth due to inherited syphilis and not produced by any other cause? The evidence in favor of an affirmative answer to this question appears to me to be so strong that I think the onus of disproof rests with the sceptics."¹³³ These views were coincided in by the majority of the gentlemen to whom they were addressed, all of them dentists and surgeons of experience and repute, and who included men so well known to the profession as Mr. Oakley Coles, Mr. Samuel Cartwright, Mr. Charles Tomes, and others.

¹³¹ Author of the section on "Surgery of the Teeth" incorporated in Bryant's Surgery.

¹³² Proceedings of the Odontological Society of Great Britain, vol. ix., 1877, pp. 238, 239. In the same journal for 1875, vol. vii. p. 17, Langdon Down says that whenever he has discovered syphilitic teeth he has "never failed to find confirmatory evidence of the syphilitic history of the case."

¹³³ He says elsewhere (Bryant's Surgery, 3d Am. ed., p. 429) that the most characteristic change in these teeth is "the lessened breadth of the cutting edge as compared with that of the neck, the vertical groove on their anterior face being often absent, and the notch on their cutting edge not being an absolutely constant feature, and being also subject to obliteration through wear."

It may be considered as well established, then, that these peculiar teeth—stunted, abnormally narrow at the cutting edge, crescentically rounded with the convexity upward, and the surface inclined upward and forward instead of backward as in normal teeth, widely separated, but converging at their lower edges—are pathognomonic of hereditary syphilis.¹³⁴ They are often described as pegged, having been likened to a row of pegs stuck in the gums. This appearance is due to the fact that they are shortened, often projecting not more than half the normal distance from the gum, and are also widely separated; which abnormalities often affect the adjoining teeth as well, and sometimes the entire dentine. It has been asserted that other specific peculiarities are to be found associated with those of the incisors, and Mr. Moon describes as characteristic, and figures¹³⁵ small dome-shaped first molars with suppressed angles and absence of enamel from the masticatory surfaces. He believes also that when the upper incisors are typical it is exceedingly rare for the lower incisors to be altogether unaffected.

¹³⁴ Mr. Hutchinson stated in 1877 that in spite of the fact that many years previously he had challenged any one to bring forward a patient with well-marked syphilitic teeth in whose history no evidence of syphilis could be found, none had come.

¹³⁵ Proc. of Odont. Soc. of Great Brit., vol. ix. pp. 241, 242; Bryant's Surgery, Am. ed., p. 429.

FIG. 11.

Serrations of Normal Incisors.

FIG. 12.

Syphilitic Incisors.

A mistake which I know, from observation, to be frequently made is the confusing of the normal serrations of the cutting edges of recently-erupted normal incisors with the peculiar crescentic edges of the syphilitic teeth. It seems worth while to call especial attention to this, on account of the unpleasant consequences which often follow injudicious questioning based on such supposed syphilitic phenomena. Indeed, the space which has been devoted to this subject of syphilis of the teeth is well warranted, I am sure, by the fact that not only do diagnosis, prognosis, and treatment in cases of great severity, and in both children and parents, often depend upon a recognition of these peculiarities, but that in addition to the clinical and therapeutic problems there are others the solution of which is dependent upon the same knowledge on the part of the practitioner, and which may involve reputation, marital relations, and personal honor and happiness.

The approximate cause of these peculiarities in the incisors can hardly be said to have been demonstrated. Mr. Hutchinson thought at one time¹³⁶ that they were due to a stomatitis or an alveolar periostitis, but he has since changed his mind as to that point, believing now¹³⁷ that the syphilitic tooth is the result of an arrest of development in the central or first-formed portion of the dentine. The incisors being made up of these lobes or denticles, and dwarfing of the middle one taking place, the two lateral ones fall together. This accounts at once for the small size of the tooth, its shape of an inverted truncated cone, and its crescentic edge.¹³⁸ If it were due to stomatitis, it would be more likely to be equally distributed, syphilis in its late manifestations being notably unsymmetrical; there would be no rational explanation of the involvement of one or two teeth while those on either side so frequently escaped; if it were mercurial stomatitis, the enamel too would be involved, as is not usually the case in the syphilitic teeth. It is possible that the central incisors are chiefly affected because they, with the first molars—also affected according to Mr. Moon—and the lower incisors—not infrequently involved—are the first-formed teeth.

¹³⁶ "The physiognomonical, dental, and other peculiarities by which we recognize the subject of inherited taint when advanced beyond the period of infancy are all of them the direct consequences of special inflammations from which the patient has suffered at former periods; e.g. the synechiæ and lustreless iris of iritis; the malformed teeth of periostitis of the alveolus and dental sacs; the protuberant forehead of hydrocephalus; the flattened nose of snuffles; the pale, earthy, opaque skin of cutaneous inflammation and eruption" (Aphorisms respecting Constitutional Syphilis, 1863).

¹³⁷ Proc. of Odont. Soc. of Great Britain, vol. ix., p. 248. See also ibid., pp. 241, 242, remarks of Mr. Moon; also Monthly Review of Dental Surgery, June 15, 1877.

¹³⁸ The denticle theory of formation is not necessarily opposed by the fact that there is only a single undivided pulp-cavity in these incisors. Instances of the separate formation of processes of dentinal pulp while others are being used and worn away, all of them finally to unite in a common pulp-chamber, have been observed in lower animals, as in the molar of the elephant.

The most elaborate article upon syphilitic teeth which has appeared since Mr. Hutchinson's original memoir is one by Fournier,¹³⁹ in which, after a very broad and comprehensive consideration of the subject, he arrives at the following conclusions: The hereditary influence of syphilis shows itself in the dental system in two ways, very unequal in point of diagnostic value—viz. first, by a retardation of evolution; second, by the arrest of growth and modifications of structure. The phenomena belonging to the second class may be grouped as follows: First, dental erosion. This is due to imperfect formation of the tooth, the result of a temporary stoppage in its development; but as it produces an appearance like that of worm-eaten wood, it has been called erosion, though in so far as the word conveys the idea of the wearing of a surface which has been previously normal, it is incorrect. The tooth affected with syphilitic erosion has never been normal. The different forms of erosion can be subdivided into groups according as they affect the face or the free edge or grinding surface of the tooth. Of those involving the face there are four types: Erosions en cupule, consisting of small excavations or cups in the surface of the crown; erosions en facettes, in which the surface presents a series of small planes, as though they had been filed; erosions en sillon when there is a linear excavation in the crown of the tooth in the shape of a transverse groove; and erosions en nappe, in which the whole surface is discolored, disorganized, and honeycombed.

¹³⁹ Archives de Derm. et Syph., Sept. 25, Oct. 2, Oct. 9, 1883. A translation made by the writer may be found in the Dental Cosmos for January and February, 1884.

A second group of erosions affects the free edge of the tooth, and includes the Hutchinson teeth, with several less important varieties. Dental erosions are multiple, symmetrical, maintain the same level on the crowns of corresponding teeth, and are situated at different heights on the crowns of teeth of different classes. It is evident, therefore, that they are the result of a morbid influence of a general character. There are three theories as to their etiology: (a) that they have no relation to syphilis,¹⁴⁰ but are always connected with infantile eclampsia; (b) that they are exclusively the result of hereditary syphilitic influence; and (c) that they are simply ordinary lesions originating from syphilis with marked frequency, and even in one form—the Hutchinson tooth—appearing to originate only from it. This latter view is the one adopted by Fournier himself. Continuing to group the symptoms due to arrest of growth and modification of structure, we have, second, microdontism, or dwarfing and stunting of the teeth—pegged teeth; third, dental amorphism, in which the teeth are strangely distorted or even transformed in type; fourth, dental vulnerability, or extreme susceptibility to all traumatic or disintegrating influences.

¹⁴⁰ M. Magitot, Treatise on the Anomalies of the Dental System, Paris, 1877; Clinical Studies on Erosion of the Teeth considered as a Retrospective Sign of Infantile Convulsions, Paris, 1881; Castanié, Paris, 1879, Thesis No. 384; Rattier, Paris, 1879, Thesis 569; and others.

Interstitial Keratitis.—The frequency of this form of diffuse inflammation of the cornea, and the diagnostic significance which has been so positively attributed to it—and has been as positively denied—render it of special interest to the general practitioner, who is almost certain to meet with occasional cases, and should be prepared to recognize its possible relation with other, and often graver, conditions.

It begins, commonly, as a slight, diffused haziness situated in the substance of the cornea itself, usually not far from the centre, and at first affecting only one eye. This depends at this stage on the presence of a number of little distinct dots of inflammation, limited to circumscribed, almost microscopic, areas, but later, in a few days, these coalesce, and at the end of a few weeks the whole cornea will probably have become nearly or quite opaque, looking like ground glass. There is no ulceration, and but little congestion as compared with that seen in other inflammatory diseases of the eye, although in the majority of cases there is a fulness of the ciliary vessels and a little photophobia with pains around the orbit. This condition may persist for one or two months, after which the other cornea is nearly always attacked,¹⁴¹ and is similarly affected, although the disease is apt to pass through its different stages rather faster than in the first eye.

¹⁴¹ In 91 out of 102 cases in Mr. Hutchinson's series—in 6, the left alone; in 5, the right alone (op. cit., p. 123).

When the height of the disease is reached the corneæ are nearly opaque, a bare perception of light remaining, so that the patient is just aware of the difference between its presence and absolute darkness. Then the cornea which was first involved begins to clear; this is soon followed by improvement in the other one, which in the course of a year or two results in a return to fairly good sight, although in most cases there remain a slight haziness and an abnormal expansion of the cornea.

This favorable result is much influenced by the character of the case, which is sometimes very mild from the outset, and by the thoroughness of the treatment. It is sometimes complicated with iritis, kerato-iritis, cyclitis, posterior choroiditis, secondary glaucoma, etc., in which cases of course the prognosis is most unfavorable. Even in ordinary cases it should be guarded in respect to perfect restoration of function, as clearing of the corneæ may reveal adhesions from iritis or spots of choroiditis disseminata, which could not, of course, have been previously detected. In very mild cases, however, without much evidence of involvement of the other tunics, and which have been submitted to treatment early, it is not uncommon for the cornea to regain almost perfect transparency.

The diagnosis of this condition may generally be made with ease. The ground-glass appearance in the earlier stages and the dull pink or salmon color in the more vascular stage are very characteristic. The vascularity differs from that attending other chronic forms of keratitis, granular lids, etc., in which the vessels are large and superficial, in that in the syphilitic keratitis they are much deeper and very closely interwoven, so that the effect is almost that of an ecchymosis. In other cases both eyes are not so apt to be affected, nor is the tendency to spontaneous cure so marked. The absence of ulceration and the very slight degree of accompanying sclerotic or ciliary congestion are also valuable features.

The conditions which Mr. Hutchinson has known to be most frequently mistaken for it¹⁴² are certain forms of inflammation following small-pox and very superficial ulcers in a healing stage, together with cases of vascular conjunctiva.

¹⁴² Op. cit., p. 128.

There is also said¹⁴³ to be some difficulty in distinguishing it from a form of non-syphilitic relapsing cyclitis with corneal opacities and iritis. This, however, is a disease of adults, often limited to one eye, with a strong tendency to relapse, the opacities being more abruptly defined and limited to the region near the circumference.

¹⁴³ Edward Nettleship in Hill and Cooper, op. cit., p. 267.

The chief point of interest, however, in the diagnosis of interstitial keratitis is its association with other symptoms of syphilis, upon which, for the general practitioner at least, the diagnosis will usually depend.¹⁴⁴ Mr. Hutchinson's conclusions, drawn from an analysis of 102 cases of interstitial keratitis, bear strongly upon this point, and are as follows:¹⁴⁵ A large proportion of all cases occur in patients between the ages of eight and fifteen, the disease being comparatively rare in early childhood, and still more so after adult age has been reached. He never saw it begin after the age of twenty-six. The patients presented the physiognomical peculiarities of inherited syphilis (see p. [313]). In more than half the cases the previous history, especially as regards infancy, was one of hereditary syphilis, and in many instances there was a clear history of infantile syphilis in brothers or sisters. In half the cases no questions were asked as to the existence of venereal disease in the parents prior to the birth of the child. In 29 out of the other half such disease was freely admitted. All the patients had lost in early life nearly half their brothers and sisters. Omitting miscarriages and premature births, it was found that 77 mothers had borne 547 children, of whom only 284 remained alive—an excessive rate of mortality. It appeared in the large proportion of cases in the eldest children in their respective families—a circumstance to be expected in view of the usual behavior of hereditary syphilis in families. Undoubted syphilitic lesions, such as nodes, ulceration of the palate, etc., are not infrequently associated with the keratitis.

¹⁴⁴ "To those who have not had opportunities for observation at an ophthalmic hospital I would recommend that the diagnosis should be held to be doubtful if the patient does not present the peculiarities of teeth and physiognomy which I have described, since we find that the latter are the almost invariable concomitants of the true disease" (Mr. Hutchinson, op. cit., p. 128).

¹⁴⁵ Op. cit., pp. 109-129.

Examination of large numbers of cases has, I believe, established the general accuracy of these statements; and although there is still much difference of opinion as to the exact relation between syphilis and this form of keratitis—whether, for example, it is a symptom of syphilis itself or of a cachexia frequently produced by syphilis, but often by other systemic diseases affecting nutrition—yet, on the whole, so far as I am able to judge of the question outside of its purely technical and ophthalmological relations, I think the weight of modern authority is chiefly on the side of a distinct and practically invariable relation of cause and effect between inherited syphilis and the corneal inflammation.

Mr. James Dixon,¹⁴⁶ for example, proposes to call the disease syphilitic keratitis, and says it is met with exclusively as a sequel of an inherited taint. He adds: "We may meet with some syphilitic keratitis in patients with healthy physiognomy and deformed teeth, or, still more rarely, in those with faultless teeth and the syphilitic cast of features; but to find the true form of keratitis in connection with both good teeth and good complexion is, I think, next to impossible." Many other ophthalmologists express themselves to the same effect more or less strongly. Nettleship,¹⁴⁷ Noyes,¹⁴⁸ Förster,¹⁴⁹ Macnamara,¹⁵⁰ De Wecker,¹⁵¹ and Carter¹⁵² may be mentioned as having ranged themselves upon this side. On the other hand we have Schweiger,¹⁵³ Maunther,¹⁵⁴ Sæmisch,¹⁵⁵ Soelberg Wells,¹⁵⁶ and others who are not convinced that syphilis is the sole nor even, in the opinion of some of them, the principal cause of this disease.

¹⁴⁶ Article on "Diseases of the Eye," Holmes's System of Surgery, Am. ed., vol. ii. p. 71.

¹⁴⁷ Op. cit.

¹⁴⁸ Text-book of Ophthalmology.

¹⁴⁹ Handbuch der gesam. Augenheilkunde, vol. vii. p. 186, 1876.

¹⁵⁰ Op. cit.

¹⁵¹ Ocular Therapeutics, trans. of Forbes, 1879, p. 124.

¹⁵² He even asserts the converse to be true, which is going beyond what I believe can be established in regard to the invariable connection between the two diseases. He says: "The subjects of what we call simply inherited syphilis are liable—nay, are almost sure—to suffer from a peculiar form of interstitial keratitis."

¹⁵³ "Hutchinson's view, that this form of keratitis is to be regarded as the direct consequence of congenital syphilis, has not been generally accepted in Germany" (Handbook of Ophthalmology, p. 298).

¹⁵⁴ Zeissl's Jahrbuch der Syphilis, 1875, p. 288.

¹⁵⁵ Graefe and S.'s Handbuch d. Augenheilkunde, 1875, vol. iv. p. 264.

¹⁵⁶ Diseases of the Eye, p. 138.

Probably the conclusions of Jullien¹⁵⁷ most nearly express the views of those who do not accept in its entirety the syphilitic theory of the disease. He concludes—(1st) that interstitial keratitis coincides frequently, but not invariably, with syphilitic derangement of the dental apparatus; (2) that it is associated with a feeble constitution and with malnutrition, and is thus produced indirectly by scrofula, rheumatism, or syphilis, each of which by its debilitating influence may give rise to a diathesis which favors such morbid developments.

¹⁵⁷ Op. cit., p. 1013.

As a specimen of the evidence which is produced by observers other than Mr. Hutchinson—who, as he himself observes, may be suspected of "that bias which almost necessarily warps more or less the judgment of one who supposes himself to have noted something new"¹⁵⁸—the statistics of Förster,¹⁵⁹ who apparently takes an unbiassed view of the question, may be noted: In a total of 214 cases of interstitial keratitis evidence of hereditary syphilis other than the corneal disease was noted in 146 (68 per cent.). This number was made up as follows: a, evidence of syphilis in parent, 17; b, evidence of hereditary syphilis in other members of the family, 14; c, evidence in the patient other than keratitis, 115; characteristic teeth, 69; evidences in physiognomy, bones, skin, palate, or choroid, 46. In the remaining 68 cases (32 per cent.), though inheritance of syphilis was not proved, there was, almost without exception, strong suspicion of that disease apart from the keratitis.

¹⁵⁸ Op. cit., Preface, p. x.

¹⁵⁹ Graefe and Sæmisch's Handbuch, vii., Part I.

The condition of the permanent central upper incisors was noted in 138 of the cases; of these they were typical in 73 (53 per cent.); suspicious in 34 (24 per cent.); normal in 31 (23 per cent.). In 15 cases the permanent teeth had not been cut, and in the remaining 61 the state of the teeth was not recorded.¹⁶⁰

¹⁶⁰ Note by Mr. Nettleship, Hill and Cooper, op. cit., pp. 263, 264.

This seems to me such direct and such unequivocal clinical evidence that it is safe to say, as of the question of syphilitic teeth, that the burden of disproof rests with the doubters, and that we may venture the assertion that interstitial, diffuse, or parenchymatous keratitis is a symptom of inherited syphilis, and that the unmistakable presence of the former disease is sufficient proof of the existence of the latter.

Syphilis of the Nerve-Centres and Nerves.—Until a comparatively recent period our only guide to the course and progress of the nerve diseases of inherited syphilis was to be found in analogy. We knew, for instance, that in acquired syphilis three forms of cerebral disease could be recognized in a general way—one characterized by sudden attack of paralysis, in which the lesion was usually thrombosis from specific endo-arteritis; one in which the symptoms of brain tumor were present, and in which gummata were the cause of the difficulty; and one in which pain, headache, and various functional or convulsive disturbances—chorea, epilepsy, paralysis of single nerves, etc.—were the customary phenomena, and in which periosteal, meningeal, or neuroglial thickenings constituted the pathological basis. The last two are often intermingled both symptomatically and histologically.

Heubner¹⁶¹ divides cerebral syphilis into three groups, two of which very closely resemble those I have described. In one, however, he includes both the general physical disturbances, incomplete paralysis, and final coma characteristic of tumor and the epileptiform attacks so often due to peripheral or meningeal irritation. This combination is explained by the results of his autopsies, which disclosed in 26 cases in which these symptoms were conjoined a gummous growth in the pia mater of the convexity of one of the cerebral hemispheres, either limited and superficial or involving more or less of the cortex and forming a distinct tumor. The epileptiform attacks were present in 19 out of these 26 cases, while in 20 other cases where the growth was limited to the white substance at the base of the brain they were present only twice. This second form is the apoplectic, followed by general hemiplegia, and depending on disease of the cerebral arteries. His third division is a very ill-defined one, depends much for its limitations upon subjective symptoms, and is of no special interest as applied to the subject of inherited syphilis.

¹⁶¹ Ziemssen, vol. xii.

Althaus¹⁶² also makes three divisions, two of which are as follows: (1) Cerebral tumor—a gumma either hard or soft. There are then nocturnal headache, sleeplessness, epileptiform attacks, the various phenomena produced by involvement of the cerebral nerves, etc. (2) Disease of the arteries, apoplexy, or softening, followed by hemiplegia.

¹⁶² Medical Times and Gazette, Nov. 10, 1877.

Hutchinson makes a similar division of lesions and symptoms,¹⁶³ and the observations of Jaksch, Wilks, and Hughlings Jackson more or less closely coincide with this general classification.

¹⁶³ Ibid., Feb. 17, 1877.

Now, in spite of certain striking differences—more apparent than real, however—between inherited and acquired syphilis as regards cause, duration of stages, etc., the essential pathological changes are the same. When syphilis in its later periods attacks the brain or spinal cord or nerve-trunks or vessels of a foetus, it proceeds just as in the adult, the same characteristic accumulation of cells taking place and setting up an arteritis or a meningitis, thickening the sheaths of nerves, or constituting a pericranial node or a gumma according to their number and their situation. We would accordingly expect to find in subjects of inherited syphilis manifestations closely allied to those observed in the adult; and the observations of Barlow,¹⁶⁴ Graefe,¹⁶⁵ Jackson,¹⁶⁶ Heubner,¹⁶⁷ Dowse,¹⁶⁸ and Hutchinson,¹⁶⁹ though comparatively few in number, have already demonstrated the correctness of this supposition.

¹⁶⁴ Trans. of Path. Soc. of London, vol. xxviii., 1877.

¹⁶⁵ Archiv für Ophthalmologie, Bd. 1, Ab. i.

¹⁶⁶ Journal of Mental Science, Jan., 1875.

¹⁶⁷ Ziemssen, vol. xii.

¹⁶⁸ Syphilis of the Brain and Spinal Cord, London, 1879, chapter on "Hered. Syph.," p. 67.

¹⁶⁹ Med. Times and Gazette (? Feb. 17, 1877).

We find, thus, that in these patients meningitis, growths, and arterial disease constitute the three clinical divisions of the disease which have thus far been distinctly differentiated, and the reported cases, with or without autopsy, fall naturally into these classes.

These cases are naturally few, and to make them absolutely reliable it is necessary to have unmistakable evidence of hereditary syphilis in some other form and the demonstration of syphilitic lesions at an autopsy. The case of Dowse, however,¹⁷⁰ includes these requirements. A child twelve years of age, of syphilitic parents, with a history of coryza, sore eyes, and a tubercular syphilide, was attacked with epilepsy, diplopia, facial paralysis, etc., and finally died. At the autopsy three gummatous growths of the surface of the brain were found, and the vessels of the base were found to have undergone the special changes described by Heubner. Their lumen was in some places nearly occluded by an accumulation of spindle-shaped cells between the tunica fenestra and the epithelial lining; and interspersed with them, but particularly in the muscular and adventitious coats, were to be seen enormous quantities of round cells which in many parts seemed actually to replace the normal structures. Dowse's other cases are not at all conclusive in their clinical histories; even the diagnosis was not established by autopsy.

¹⁷⁰ Op cit., pp. 71-75.

Barlow's two cases were both very young children, and are extremely convincing.¹⁷¹ A child four months old, with snuffles, serpiginous ulcers, etc., and with a syphilitic father, had epileptiform attacks, followed by laryngismus, carpo-pedal contraction, and changes in the choroid. She died aged about fifteen months, and the autopsy disclosed thickening of the pia mater, evidently not tubercular, and changes in the arteries, which in the gradual narrowing of the lumen of the vessel, the absence of ulceration or disintegration or calcification, and the continuity and extent of the cell-proliferation are as different as possible from ordinary atheroma, but correspond precisely with the description of Heubner's cases, which were undoubtedly the subjects of acquired syphilis.

¹⁷¹ Transactions of the Pathological Society of London, 1877, vol. xxviii. pp. 287-291.

In the second case the symptoms were associated chiefly with the cranial nerves. These were nystagmus, paresis of facial muscles, laryngeal spasms, etc. He died at the age of fifteen months. The vessels of the base were extensively diseased as in the other case, and the fourth, fifth, sixth, seventh, and eighth pairs of nerves were smaller by a new growth of a gummatous nature which had produced almost entire atrophy of the nerve-cylinders. There were cicatrices of the liver and spleen. Cases in which the diagnosis rested upon the history, upon the co-existence of undoubted syphilitic symptoms, and upon improvement under specific treatment are by no means rare.

Berkely Hill reports¹⁷² a typical case of syphilitic epilepsy. A girl aged nine was the first child of the family that had lived, two having previously been born dead. She presented characteristic teeth, traces of choroiditis, and, while under treatment, suffered from both keratitis and iritis. Her first fit was when she was four years of age, and the attacks had recurred frequently since that time. The convulsion was confined to the left side. The left arm was very weak, the weakness having come on gradually, and being especially great in the extensors of the wrist. Under specific treatment the attacks ceased entirely. In this case there was certainly organic disease, probably a gumma on the surface of the right hemisphere.

¹⁷² Op. cit., p. 253.

Other cases reported by the same author, numerous instances of nervous troubles in inherited syphilis reported by Hughlings Jackson,¹⁷³ Fournier,¹⁷⁴ Henoch,¹⁷⁵ and Hutchinson,¹⁷⁶ warrant the statement, then, that the nervous diseases of inherited syphilis fall into the same general category as those of the acquired disease; that they may appear at any age, from three or four months to that of puberty; that they depend for their production upon a cell-proliferation which, according to its locality, results in the development of new growths, the production of localized meningeal inflammations, or the obstruction and inflammation of arteries; and that the symptoms are those usually associated with such pathological changes, the diagnosis of syphilis depending chiefly on the history and the presence of other specific phenomena.

¹⁷³ Journal of Mental Science, Jan. 8, 1875; Transactions of St. Andrew's Med. Graduates' Ass., vol. i., 1868.

¹⁷⁴ Annales des Derm. et Syph.

¹⁷⁵ Nouveau Dict. de Méd. et Chirurgie, p. 885.

¹⁷⁶ Op. cit.

The prognosis is more unfavorable than in similar cases in acquired syphilis, the meningeal and vascular lesions being the most frequent and showing themselves very obstinate even under careful treatment. Spinal troubles, chorea, and idiocy have been attributed to inherited syphilis, but must occur with great rarity.

Hill¹⁷⁷ reports a case of a child aged five years who had been hemiplegic since he was two and a half years old, and who developed paralysis of the flexors of the ankle in each leg.

¹⁷⁷ Op. cit., p. 254.

Keyes reports a case of a boy five years of age, with nodes on tibiæ and other signs of inherited syphilis, who had two attacks of paraplegia.

Hill reports a case of imbecility associated with inherited syphilis, but there is no evidence that it was other than a coincidence.

Fletcher Beach found not more than 1 per cent. of syphilitic children in the Dareult Asylum, and Mr. Mercier could only trace syphilis in 5 out of 220 female idiots, probably imbecile from birth.¹⁷⁸

¹⁷⁸ Ibid., p. 255.

Hughlings Jackson only found 1 case of inherited syphilis among 80 cases of chorea. The most carefully recorded cases of the latter affection associated with hereditary syphilis are two reported by Alison.¹⁷⁹

¹⁷⁹ American Journal of the Medical Sciences, July, 1877.

Syphilis of the Spleen.—Disease of this organ in inherited syphilis is especially important from two points of view. It is a valuable aid to diagnosis, and by its size and the degree of persistence of the swelling gives an approximate indication of the severity of the case.

Attention was first called to the frequency and importance of enlargement of the spleen in early hereditary syphilis by Gee in a paper read before the Royal Medical and Chirurgical Society in 1867.¹⁸⁰ He gave the histories of thirteen children in support of the statement that such enlargement occurred in almost one-fourth of all cases of hereditary syphilis, sometimes with, sometimes without, enlargement of the liver and lymphatic glands. According to him, the degree of splenic enlargement may be taken as a sort of index of the severity of the cachexia; the majority of cases with great enlargement die, but sometimes such children survive, the spleen gradually diminishing in size as the health improves—not diminishing, however, pari passu with such improvement, but remaining for a long time "a monument of past cachexia."

¹⁸⁰ British Medical Journal, 1867, vol. i. p. 435.

Barlow,¹⁸¹ ten years later, thought Gee had rather understated the proportion of cases in which splenic enlargement occurs, he having found it in 22 out of 28 children with definite hereditary syphilis. Birch-Hirschfeld, Eisenschitz, and Tepel¹⁸² corroborate these observations, finding that the enlargement is almost invariable and that the spleen is often double its normal size.

¹⁸¹ Trans. of Path. Soc. of London, Jan. 20, 1877.

¹⁸² Quoted by Hill and Cooper, op. cit., pp. 164, 165.

Mr. W. J. Tyson has reported¹⁸³ a cure of a child born of syphilitic parents, in whom, at two years of age, the spleen extended downward three and a half inches, reaching the crest of the ilium and approaching closely to the umbilicus. The liver was not enlarged; the urine was not albuminous. He ordered mercury with chalk, one grain every morning and evening, and one grain of iodide of potassium, with ten minims of syrup of iodide of iron to an ounce of water, three times a day. Sixteen months later the spleen had become imperceptible, and three years afterward the child was in excellent health.

¹⁸³ The Lancet, Oct. 23, 1880.

The diminution of the liver under treatment appears to take place before there is any diminution in the size of the spleen.¹⁸⁴ This persistence of the latter renders it, as has been stated, a valuable diagnostic sign. In the paper already quoted from,¹⁸⁵ Macnamara and Barlow allude to this as follows: Enlargement of the liver, although it ought to be noted because it is often present in hereditary syphilis, has but little value as a confirmatory symptom—first, because the liver is proportionally large in infancy, and it is difficult to state the limit of what is actually normal; and, secondly, because other causes besides congenital syphilis lead to its enlargement.

¹⁸⁴ Barlow, British Medical Journal, Jan. 20, 1877.

¹⁸⁵ British Medical Journal, Dec. 16, 1882.

With regard to enlargement of the spleen the case is different. Gee's observation, that in the early stage of infantile syphilis some enlargement of the spleen occurs in a large number of cases, has been abundantly confirmed. Although with the subsidence of the other symptoms this enlargement often disappears, so that on post-mortem examination two or three months after there may be no trace of it, yet in a few cases it persists, and indeed sometimes increases, so as to be considerable when the other signs have quite vanished. The importance of this sign is greatest when noted early; as, for example, when the child is from two to three months old, for at that period the enlargement of the spleen due to rickets can hardly come into question.

The condition of the spleen during this period of enlargement seems to be simply that of hyperæmia, or at the most of hyperplasia, Gee's, Barlow's, and Birch-Hirschfeld's autopsies showing no evidence of new growth or of amyloid or other changes.¹⁸⁶

¹⁸⁶ Parrot reports (Le Mouvement méd., Paris, Nov. 23, 1872) two forms of splenic disease produced by inherited syphilis: 1. A simple hypertrophy, which he thinks is secondary to diffuse infiltration of the liver, obstructing the portal circulation and causing the spleen to act as a reservoir; 2. An inflammatory condition resulting in the formation of false membranes around the capsule. His explanation of the first condition is unsatisfactory, because there are numerous cases in which the spleen is enlarged without any involvement of the liver. His other observations have never been confirmed.

The cause seems to me to be in all probability the well-known effect of syphilis on the glandular system in general, and the lymphatic system in particular, to which I believe the spleen is now generally assigned. The analogy between this slow, persistent, painless enlargement preceding the cutaneous symptoms,¹⁸⁷ unaccompanied by inflammatory symptoms, unattended by any breaking down of tissue, subsiding slowly but evenly under specific treatment, and the behavior of the buboes of acquired syphilis, is certainly very striking.¹⁸⁸

¹⁸⁷ Eisenschitz, Wiener med. Wochenschrift, Nos. 48 and 49.

¹⁸⁸ A similar enlargement occurs, but much more rarely, in the secondary period of acquired syphilis. Weaver noted it in 3 out of 79 soldiers suffering from early syphilis. Wilks and Moxon report cases in which the average weight was 19 oz.

In most cases of hereditary syphilis there are evidences of disturbance of the gastro-intestinal tract. Vomiting, diarrhoea, colic, anorexia, and emaciation are well-known, but of course not at all characteristic, symptoms.

It has been supposed that the mucous membrane of the entire tract was probably, during the early period at least, and coincidently with the cutaneous eruption, in a condition of hyperæmia and irritation comparable to that of the skin.¹⁸⁹ Whether this be so, or whether it is due to associated involvement of the glandular apparatus, has not yet been determined.

¹⁸⁹ "There is surely no a priori probability that a blood disease so severe as syphilis should produce lesions on the skin, in the mouth, and in the eye only—that it should, in fact, affect all the visible parts and avoid all the concealed ones" (Mr. Hutchinson, The Lancet, Feb. 6, 1876).

Förster¹⁹⁰ has found fibroid degeneration of Peyer's patches in a syphilitic infant who died six days after birth, the glandular structure having been replaced by elevated grayish-red masses of nuclei, cells, and connective-tissue fibre. Ulcers of the intestines have been described, but appear to have had no specific characters.

¹⁹⁰ Quoted by Bumstead and Taylor, op. cit., p. 757. His observations were confirmed by Eberth, Roth, and Oser.

The pancreas has been most extensively studied by Birch-Hirschfeld, who examined seventy-three syphilitic foetuses. In thirteen of them he found enlargement of the pancreas with increased weight and density, proliferation of connective tissue, and in some cases compression—almost obliteration—of the lobules, with atrophy and fatty degeneration of their epithelium.¹⁹¹

¹⁹¹ Klebs discovered a gumma in the pancreas of a six-months' foetus.

The peritoneum has been described by Simpson, Von Baerensprung, and others as occasionally invaded in early hereditary syphilis. There seems to be no evidence that it is ever directly affected, the cases in which death occurs from peritonitis being due usually to trouble connected with the liver or spleen.

Syphilis of the lung, originally described by Depaul and Virchow, has been carefully studied also by Förster, Robin, Lorain, and Cornil, from whose description¹⁹² of the pathology of the condition I condense the following: In the syphilitic foetus born before term, in the syphilitic child born dead at full term, and in the syphilitic children who live a few days, there are found at the autopsy, in the lungs, nodules or tumors, usually superficial, sometimes deep, hard, isolated or in groups, pink, gray, or red in color, with scattered whitish or yellowish points. Their size varies from a pea to a small walnut. They represent a portion of the lung more or less considerable in a state of special lobular hepatization. Sometimes a whole lobe is involved. The affected portion is very dense and covered with thickened inflamed pleura. The lesions are those of chronic pneumonia. The interlobular connective tissue enters into proliferation and presents a large quantity of embryonal cells; the alveolar walls are thick, while the narrow alveoli are livid, and even filled by epithelial cells, which are of the pavement form in contact with the walls, round in the centre of the alveoli. As the process progresses the epithelial cells become fatty, degenerated, and subsequently broken down and absorbed, while the embryonal interalveolar tissue rapidly organizes into fibrous tissue. Thus results a small fibrous tumor, in which a gumma may ultimately develop.¹⁹³

¹⁹² Syphilis, trans. of Simes and White, Philada., 1882, p. 404.

¹⁹³ Cornil and Ranvier's Path. Histology, Am. ed., 1880.

It is impossible to confound this syphilitic pneumonia with tuberculosis. The granulations of tubercle are never congenital.

The fact that in syphilitic interstitial hyperplasia the change begins in the interlobular connective tissue and around the interlobular vessels, at first consisting of small spindle-shaped and roundish cells which quickly develop into connective tissue, and the fact that blood-vessels are freely produced among the fibres of this new tissue, seemed, in the estimation of those pathologists who took part in the discussion on visceral syphilis in 1877, to constitute its most distinctive feature.¹⁹⁴

¹⁹⁴ Trans. of Lond. Path. Soc., vol. xxviii.—views of Green, Jones, Greenfield, Moxon, and others.

Gummata in the lungs of children suffering from inherited syphilis have been described by a number of writers. They appear likewise to begin in the walls of the blood-vessels or the bronchioles. They differ from tubercular nodules in being few in number—not more than half a dozen usually—and are generally confined to one lung.

The condition of the arteries in the few cases in which they have been noticed as affected by inherited syphilis was precisely similar to that found by Heubner in the arteries of adults.¹⁹⁵

¹⁹⁵ See Cornil, op. cit., p. 305.

The symptoms are very variable; new-born children often die asphyxiated in a few days. If they live longer, the disease develops into a more generalized broncho-pneumonia.

Syphilis of the Larynx.—The hoarse cry of the new-born infant so characteristic of hereditary syphilis depends upon the presence of hyperæmia, of mucous patches, or even of extensive ulceration. I am inclined to think that the first is the more common, as if it were otherwise cases of death from oedema glottidis or other forms of laryngeal obstruction would be oftener met with. When ulceration does exist it is generally, but not invariably, secondary to pharyngeal ulcers.¹⁹⁶

¹⁹⁶ See synopsis of six cases of George M. Lefferts, reported in Bumstead and Taylor, op. cit., p. 754.

Bronchial catarrh, giving rise to cough, and sometimes to considerable embarrassment of respiration, is a not infrequent complication of laryngeal syphilis.¹⁹⁷

¹⁹⁷ Schnitzler, Die Lungen Syphilis, etc., 1880, S. 41.

Later troubles of the larynx in connection with inherited syphilis have not yet been carefully enough studied to warrant us in drawing any distinction between them and the usual symptoms seen in the acquired disease.

Syphilis of the testicles has been studied by Henoch,¹⁹⁸ Cornil,¹⁹⁹ Parrot,²⁰⁰ Hutinel,²⁰¹ North,²⁰² Bryant,²⁰³ and others. It is found to consist of a true interstitial orchitis, very closely resembling that seen in the syphilitic testicles of adults. Hutinel's investigations, based on ten cases, showed the testicles slightly enlarged and harder than normal, the scrotum pendent, the epididymis normal, the tension of the tunica vaginalis and tunica albuginea slight. The basis of the lesion is in a collection of small round embryonal cells resembling lymph-cells, arranged in the connective tissue around the arterioles which come from the tunica albuginea. This may be accompanied by a more or less marked diffused interstitial orchitis, or there may be only a thickening from the new formation of small round cells on the connective tissues of the testicles. Cornil found the seminal ducts separated by numerous round or fusiform cells. The disease usually occurs at from two months to three years of age; both testicles are generally involved, and are enlarged, hard, inelastic, and frequently nodulated. Mercurial treatment generally causes a marked improvement unless the inflammation has already resulted in the development of a new fibroid formation, in which case it would be likely to remain unaffected by any form of treatment. Inunctions with diluted mercurial ointment, iodoform, etc. are useful adjuvants.

¹⁹⁸ Schmidt's Jahrbuch, 178, No. 4.

¹⁹⁹ Op. cit., p. 420.

²⁰⁰ Rev. mens. de Méd. et de Chir., Paris, Feb., 1878.

²⁰¹ Ibid.

²⁰² Med. Times and Gaz., Lond., 1862, vol. i. p. 403.

²⁰³ Ibid., Dec., 1863.

The kidneys are not infrequently involved in inherited syphilis. Parrot reports the pathological change to consist of a proliferation of small round cells in the intertubular connective tissue, followed by contraction, obliteration of the tubules, and degeneration of their epithelium.

Bradley has reported²⁰⁴ the case of a child aged four months in whom a well-marked syphilitic eruption and an attack of acute Bright's disease were coincident. Mercurial treatment for three weeks cured both.

²⁰⁴ British Med. Journ., Feb. 4, 1876.

Coupland has reported two cases of parenchymatous nephritis associated with inherited syphilis, but advances no proof that it was not an accident. Gummata have been from time to time found in the kidneys of very young children who have died from their effects and from other visceral lesions due to syphilis.²⁰⁵ Cases of enlargement, of fibroid, fatty, and gelatinous degenerations of the suprarenal capsules, have been recorded.

²⁰⁵ See discussion in Clinical Soc. of London, Jan., 1880; "Remarks on Visceral, and especially on Renal, Syphilis," by Barthelémy, Annales de Derm. et Syph., April, 1881.

The thymus gland is occasionally found in syphilis to have undergone alterations claimed by Dubois, Depaul, and others to be syphilitic in their nature, but ascribed by Parrot simply to degenerative changes due to malnutrition. The gland does not appear to undergo any marked alteration in size, color, or consistency, but is found after death to contain a small quantity of purulent matter.

The tendency of syphilis is certainly not, as a rule, to the formation of pus. Nearly all the lesions we have studied with the exception of breaking-down gummata have consisted in various forms of cell-proliferation or accumulation, and not in the formation of abscesses, and it is not probable that this is an exception. I doubt very much the syphilitic character of these changes.²⁰⁶

²⁰⁶ Lancereaux believed that it was due to the breaking down of a gummy deposit, but that seems to be entirely hypothetical, none having been discovered. Weisflag (quoted by Bumstead) arrives at the following conclusions after studying the lesion and the literature of the subject: 1. This thymus abscess does exist. 2. When associated with other signs of congenital syphilis it indicates that the father or mother of an infant suffers or has suffered from syphilis. 3. It is possible, but not proved, that this affection may exist in children in whom there are no symptoms of syphilis, but its existence renders the diagnosis of hereditary syphilis probable, even if the disease of the parent is not proved. 4. Such is the great similarity in the appearance of pus and of the secretion of the thymus that they cannot always be distinguished.

THE DIAGNOSIS AND PROGNOSIS OF INHERITED SYPHILIS.—In reviewing the general course of a case of inherited syphilis it seems evident that the differences between it and the acquired disease which have been so much dwelt upon are apparent rather than real.²⁰⁷ The primary stage is of course missing, and on any theory of the essential nature of syphilis this is readily comprehensible. Whether the chancre is the first symptom of a constitutional disease, or, as I believe to be the case, is the simple accumulation at the point of original inoculation of the cells which constitute the syphilitic virus—or are at any rate its carriers—it would naturally be in the first case undiscoverable, in the second nonexistent.

²⁰⁷ "That the noteworthy differences between chancre-syphilis and the inherited disease are to be interpreted by considerations of the tissues of the growing child and the adult, is made very probable by what is observed when a mother near the end of pregnancy becomes infected with primary disease. In such a case the foetus nearly full grown acquires the disease, without a chancre, directly from the maternal blood. It is acquisition, not inheritance, for at the date of conception both the paternal and maternal elements were free from taint, and during the first six, seven, or even eight months of intra-uterine life the foetus remained healthy. Yet, as I have proved elsewhere by citation of cases, syphilis obtained in this peculiar method resembles exactly that which comes by true inheritance, and not that which follows a chancre. This important fact goes, with many others, in support of the belief that the poison of syphilis remains identical, however obtained, and that the differences which are so patent in its manifestations are due to differences in the state of its recipient" (Mr. Hutchinson, article on "Transmission of Syphilis," Brit. and For. Med.-Chir. Rev., Oct., 1877, p. 475).

"It is not true that the diversity of symptoms presented by infants authorizes us to admit a congenital and an hereditary syphilis. Whatever the mode of infection, it is impossible to make this distinction" (Ricord, note to John Hunter's Works, 1883).

The secondary stage, characterized in the acquired form chiefly by lymphatic engorgement and symmetrical, widely-spread, polymorphic cutaneous and mucous eruptions, and pathologically by a marked tendency to the proliferation of certain new small round nucleated cells, upon the presence of which depend all the manifestations of the disease, is in inherited syphilis strictly analogous. Eruptions of the same character make their appearance, differing only in minor points, as in a greater tendency to become moist or ulcerated, due to the more delicate texture of the infantile epidermis. To the same cause must be assigned the macroscopic peculiarities of the only syphiloderm said to be peculiar to infantile syphilis—pemphigus—which has been shown, however, to have a papular basis, and in that way to conform to all the other secondary eruptions.

The lymphatic engorgement either exists in the infant as in the adult or has its analogue in the enlargement of the spleen and liver—especially the former, which is almost as constant a phenomenon as is general glandular enlargement in acquired syphilis. The same pathological changes occur, the same infiltration of cells producing, according to their situation, papular, pustular, or mucous patches, or inflammation of such structures as the iris, choroid, or retina.

The tertiary stage, except in the fact that its phenomena may appear unusually early and may be commingled with those of the secondary period,²⁰⁸ does not widely differ in the hereditary from that of the acquired disease. It affects the same tissues, results in the same pathological formations, and is preceded by the same period of latency or quiescence of variable duration. There is no reliable evidence with which I am familiar to show that in this stage inherited syphilis is either contagious or transmissible—another point of close resemblance between the two varieties under consideration.

²⁰⁸ This is by no means unknown even in the acquired form; frequent examples of it have been recorded, and it can be readily explained either on the theory of relapses in parts previously diseased (Hutchinson), or on that of obliteration of lymphatic trunks and accumulation of nutritive waste (Otis).

In considering the question of diagnosis, therefore, we have an excellent guide in the fact that the disease conforms in most respects to the general laws of acquired syphilis, and that our knowledge of the latter affection will be a valuable aid to recognition of the former.

The chief elements of diagnosis and prognosis of inherited syphilis in its various stages may then be summarized as follows:

A history of syphilis in either parent is important just in proportion to the shortness of the interval between the time of infection and the date of conception. In other words, the shorter that interval the more likely (a) that the child will have syphilis, (b) that it will have it in a severe or fatal form. If the mother has been syphilitic and the father healthy—which is rare—it is perhaps more likely that the child will be diseased than when the reverse is the case. If both parents were syphilitic at or before the time of conception, the probability that the disease will be transmitted, and in a severe form, is much increased. There is no evidence to show that inheritance from one parent results in a graver variety of the disease than when it is derived from the other.

A history of abortion or miscarriage on the part of the mother should have weight in the determination of any given case, and if such accidents have been very frequent their diagnostic importance is greatly increased. The loss of elder brothers or sisters and the causes of death, with the precedent symptoms, should be carefully inquired into. The nearer either of these occurrences—abortion or death of elder children, if there is a fair presumption that they were due to syphilis—has been to the birth of the patient in question, the greater the likelihood that the latter has been infected.

Upon examining the product of abortion or stillbirth the most easily observable symptoms will be those of the skin. Maceration and elevation of the epidermis into bullæ are in themselves hardly characteristic, though they may—especially the latter—be regarded as suspicious. If the cutaneous lesions are, however, distinctly papular or pustular or ulcerative, or if the bullæ have all the characteristics of syphilitic pemphigus, the diagnosis is assured.²⁰⁹

²⁰⁹ "It is probable that very early abortions are less rare than statistics indicate, but are often unsuspected."

"It is impossible to demonstrate the existence of syphilitic lesions in foetuses expelled during the first months of pregnancy. Later, the signs which have the greatest value are the lesions of the epiphyses of the long bones. When the foetus has nearly arrived at full term, and is not macerated, visceral and cutaneous lesions may be observed. According to Mewis, the skin eruptions cannot be seen before the eighth month, and are only recognizable on foetuses whose death has been very recent or who are born living. Pulmonary lesions may be determined at the end of the sixth month. Those of the pancreas are met with in about half the foetuses which perish a little before or a little after birth. The lesions of the liver, the spleen, and the bones may be recognized even in macerated foetuses, this frequency increasing from month to month" (Nouv. Dict. de Méd. et Chir., vol. xxxiv. p. 864).

The most distinctive symptom—one which may really be considered as pathognomonic, is, however, the inflammation of the diaphyso-epiphysial articulations, with or without their disjunction. Distinct enlargement of the spleen or liver, and arachnitis with hydrocephalus, are valuable diagnostic points, and the presence of gummata—not very infrequent—would of course be conclusive.

At birth the syphilitic child may be small, stunted, emaciated, weazened, senile in appearance; this would properly give rise to suspicion, but may be associated with any disorder of nutrition on the part of child or mother. It may also disclose cutaneous or mucous eruptions evidently specific in character. The most common of these at this early date is the bullous eruption affecting the palms and soles, sometimes distributed over the whole body, and, as it indicates a feeble resistance of the tissues to the tendency to exudation and cell-growth, is usually a precursor of an early and fatal termination. In any event, marked symptoms at time of birth render the prognosis highly unfavorable.

It is quite as common, however—perhaps more so—for the subject of hereditary syphilis to give no evidence of the disease at birth, but even to appear healthy and well-nourished. In such cases the first symptoms of the disease appear, on an average, in from six weeks to two or three months, and consist principally of coryza (snuffles), hoarseness of voice, and syphilodermata. The latter may be macular, papular, pustular, or bullous. They are usually polymorphous, irregular in shape, dark coppery-red in color, with sometimes a glazed or crusted, but oftener a moist or ulcerating, surface, with a strong tendency to coalesce into large patches, or to form irregular serpiginous ulcers, or to take on hypertrophic growth and develop into condylomata. Eruptions which are squamous and are situated about the mouth and chin and on the body, the legs, or the soles of the feet, though exceptional, are of more value than those on the nates, where the results of irritation from urine and feces may closely simulate syphilodermata.

Mucous patches on the tongue, cheeks, tonsils, and pharynx are common, often extending to the larynx, increasing the hoarseness, and to the nasal cavities, aggravating the snuffles. Both of these occurrences, by interfering with the respiration of the child and rendering its nursing interrupted and insufficient, greatly add to the gravity of the case. Enlargement of the spleen (common), enlargement of the liver (less so), and iritis (rare), may be mentioned among the phenomena of this stage, often associated with the skin eruptions.

About the time of the subsidence of the rash there may be developed the specific inflammation at the junction of epiphyses and diaphyses which produces a swelling of the long bones near their ends. The child will be noticed to cry a little when, for example, the wrist or elbow on one side is washed, and not to use these joints as much as the corresponding ones on the other side. The parts are not hot, only slightly tender, and as yet there is but little swelling. Later, the droop and the disuse of the affected limb become more noticeable and simulate infantile paralysis. There is, however, no wasting, no alteration of reaction by faradism, no real loss of power, so that the term pseudo-paralysis is an appropriate one. In a week or two similar symptoms will occur in the bone on the opposite side, and finally the ends of all the long bones may be affected; ordinarily the elbows, wrists, knees, and shoulders are the joints involved. Suppuration is rare, disjunction of the epiphysis from the diaphysis common. Recovery is apt to take place spontaneously within a month. The associated changes are chiefly endosteal at the junction of the shaft with the epiphysis, but there is also a little periostitis or perichondritis, which is the principal cause of the external swelling. Moderate deformity may ensue.²¹⁰

²¹⁰ For the diagnosis from rickets see p. [290].

Similar changes occurring in the cranial bones give rise to what has been called the natiform skull. During the first year it is very common for syphilitic children to develop a number of lenticular swellings on the cranium, which appear symmetrically around the anterior fontanel, but at a little distance from it; i.e. one on each frontal and one on each parietal bone. These are said to be "bossed." They are at first circumscribed, and in a child nine or ten months old often measure three-quarters of an inch to an inch in diameter. They are at first circular, afterward more irregular, and finally tend to organize, becoming diffused and massive and causing a permanent thickening of the skull.

These symptoms which have been described are the prominent ones occurring during the first six or eight or twelve months of life. If they do not manifest themselves before the eighth month, it is highly probable, even in a case with a syphilitic parental history, that the child will either escape altogether or that the secondary stage has been very slight and altogether intra-uterine and unattended with noticeable phenomena. If during this first year the child's cachexia is marked, if there are any intercurrent diseases, if the symptoms show themselves early, if the nasal or laryngeal affection is severe, if the eruptions are markedly bullar or pustular or ulcerative, if the enlargement of the spleen is great or the osseous lesions precocious or grave, and if, especially, there is any intermingling of tertiary symptoms, gummata, nodes, etc.,—the prognosis will be unfavorable.

From adolescence on through adult life the diagnosis of inherited syphilis will depend on the following points: First, of course, the history of parental or of infantile syphilis, or of both. Then a group of physical and physiognomical peculiarities, which are not definitely characteristic, and are of little value when taken separately, but are of considerable importance when all or a majority are present in any given case. These are low stature or puny development proportionate to the severity of the intra-uterine and infantile symptoms; a pasty, leaden, or earthy complexion,²¹¹ a relic of previous syphilodermata, probably also a result of malnutrition; a prominent forehead, bulging in the middle line at and within the frontal eminence, and due either to thickening of the skull or to a previous arachnitis and hydrocephalus before the ossification of the fontanels; a flat, sunken bridge to the nose, due to the coryza of infancy extending to the periosteum of the delicate nasal bones, and either interfering with their nutrition or partially destroying them; dryness and thinness of the hair, with brittleness and splitting of the nails; synechiæ and dulness of the iris (rare); ulcerations of the hard palate;²¹² and periosteal thickenings or enlargements of the shafts of the long bones near the ends, or slight angular deformity, results of the osteo-chondritis of infancy.

²¹¹ Trousseau (Clinical Lectures, vol. ii. p. 588, Philada., 1873), after calling attention to this peculiar hue of the face, says: "It not unfrequently happens that the physician, taught by long familiarity with this appearance, will almost at once diagnose syphilis after having simply seen the child's face, although the peculiar hue can be but vaguely described in words. The visage presents a special shade of bistre; it looks as if it had been lightly smeared with coffee-grounds or a very dilute aqueous solution of soot. There is neither the pallor, the icteric hue, nor the straw-yellow tinge of skin seen in other cachectic affections; the tinge is not nearly so deep, but is almost like that of the countenance of a recently-delivered woman, and either does not extend at all, or only partially, to the rest of the body. I know no disease except syphilis in which a child's skin has this peculiar color; and consequently, when it is well marked, it has more diagnostic value than any other symptom."

²¹² Mr. Oakley Coles reports (Proc. of Path. Society of Great Britain, vol. vii. p. 5) several cases of inherited syphilis in which there was wide separation of the jaws in the median line. In one family one member had typical teeth and wide separation; three others had the same separation, but not the characteristic teeth. It was suggested that in such cases the teeth were in size far below the average, and that the condition was that often observed where the jaws are in development in excess of the teeth which they contain. I. E. Atkinson details some interesting cases of this lesion in late hereditary syphilis, and attributes to it considerable diagnostic importance (American Journal of the Medical Sciences, New Series, vol. lxxvii., Jan., 1879, p. 71).

A much more valuable group of symptoms, however, are the following, which are mentioned in the order of their importance, any one of the first three being almost or quite conclusive:

Dwarfed permanent median upper incisors, broader at the top than at the cutting edge, which is crescentically notched, separated by an undue interval and converging toward each other.

Evidence of past or present interstitial keratitis—a dusky and thin sclerotic in the ciliary region and slight clouds here and there in the corneal substance, there being no scars on its surface—or of disseminated choroiditis; patches of absorption especially around the periphery.

A radiating series of narrow cicatricial scars extending right across the mucous membrane of the lips, or a network of linear cicatrices on the upper lip and around the nostrils, as well as at the corners of the mouth and on the lower lip.

Periosteal nodes on one or many of the long bones; sudden, symmetrical, and complete deafness, without otorrhoea and unattended by pain or other subjective symptoms.²¹³

²¹³ In a few instances there has been noticed an arrest of sexual development; in one case of Hughlings Jackson's there was such an entire absence of all sexual characteristics that it was supposed that the ovaries had been destroyed by syphilitic inflammation in early life.

Late or tardy hereditary syphilis is rarely dangerous to life. The prognosis is almost unvaryingly favorable unless some grave visceral complication, such as interstitial pneumonia, gummata of the brain, liver, or kidney, or meningeal and periosteal inflammation within the cranium, should occur.

TREATMENT.—The prophylactic treatment, or that directed to the health and sexual relations of the parents previous to conception, has already been sufficiently considered. That of the mother during pregnancy, after having conceived from a syphilitic husband, or having had antecedent syphilis, or having contracted it by direct contagion subsequent to impregnation, is simply that of acquired syphilis in either adult or child. Mercury in its full physiological dose is the drug indicated. It may not be amiss to combine with it iodide of potassium in moderate doses, but the practice of employing the latter to the exclusion of the former is both theoretically and clinically unsound. Care should especially be taken to give it in such a manner, either by inunction or vaporization or so guarded with opium, that it will not produce any irritating effect on the intestinal canal, the sympathy between which and the uterus may, in the event of a strong purgative action being set up, lead to an abortion.²¹⁴

²¹⁴ "In respect to prophylaxis as applied to infants, all chances of infection should be entirely removed whenever constitutional symptoms exist or the nature of the primary symptoms renders them probable. Our caution should be carried still farther, and in the absence of all appreciable symptoms we should assure ourselves by the antecedents, so far as possible, that the parents are not under the influence of a syphilitic diathesis; in which case they may give birth to infected infants until appropriate treatment shields the latter from infection. With still stronger reasons, when the mother during pregnancy is affected with primary syphilitic symptoms of such a character as to give rise to secondary symptoms, or if the latter already exist, we should hasten to cope with them, and, far from regarding pregnancy as a contra-indication to treatment, should recollect that it generally prevents the disease in the infant, and when skilfully administered obviates the frequent abortions which syphilis excites. When primary symptoms have been contracted by the mother a short time before delivery, since the infant may be infected in its passage into the world, the same course should be followed with it as with a person who has just exposed himself to an impure connection" (Ricord, note on prophylaxis of venereal disease appended to his edition of John Hunter's Treatise on Venereal, Philada., 1853, p. 481).

As we have seen that the pathology, the stages, and the general course of hereditary syphilis are all closely related to or identical with the same phenomena in the acquired disease, and so know that they both depend upon the same ultimate cause, whatever that may be—a virus, a fungus, or a degraded cell—it follows that the same principles should govern us in the treatment of the one as in that of the other.

We know from clinical experience that mercury exercises an almost controlling influence over the secondary manifestations of acquired syphilis, whether by acting as a true antidote or as a tonic, or by virtue of its property of hastening destructive metamorphosis and thereby facilitating the absorption or elimination of new cell-growths. We know also that iodide of potassium, probably by virtue of its powerful stimulating influence on the lymphatic system, has an equal power over the tertiary growths, which by their pressure upon or situation in important tissues or organs may be so destructive. There is no reason, therefore, by analogy why these drugs should not, comparatively speaking, be equally beneficial in hereditary syphilis; and such is, indeed, found to be the case. In the latter affection, however, there are two elements which should modify the treatment somewhat, and must be taken into consideration. These are—1st. The existence of a more or less profound cachexia influencing all the nutritive and formative processes, and in itself, entirely apart from any definite specific involvement of vital organs, threatening life. 2d. The not infrequent occurrence during the secondary period of symptoms—notably gummata—belonging to the tertiary stage.

The first indication is met by making the treatment from first to last not only antisyphilitic, but also supporting or even stimulating; and with this object in view especial attention should be paid to nutrition. It may be stated, axiomatically, that for every reason, whenever it is within the bounds of possibility, the nurse of a syphilitic child should be its mother. To her it is harmless—to every other woman, not already syphilized, it is in the highest degree dangerous. Space will not permit me here to discuss the medico-legal aspect of the interesting question as to relations between such children and the outside world, especially as represented in their nurses. It will suffice to say that it is criminal and legally punishable to induce any healthy woman to act as wet-nurse to a syphilitic child unless she does so with a full knowledge of the risks she runs in undertaking that function. In the rare cases where with such information she still consents to suckle the child a written statement of the facts of the case should be signed by her, with the proper legal formalities, for the protection of the physician and the family.

If the mother has died or on account of ill-health is unable to nurse her child, and if no wet-nurse willing to enter the above agreement can be obtained, the possibility and propriety of obtaining one who has already had syphilis must next be considered. This idea to many parents seems revolting, but will naturally be less so to those who have themselves had the disease, and is, besides, so almost vitally important to the child that no hesitation should be felt about making the suggestion. If it is accepted, and if there is any opportunity for making a selection, it may be said that the more robust the present condition of such a nurse, and the more remote the date of her syphilis, the better will be the chances of the child.

If neither mother nor wet-nurse can be had to suckle the child, it must be fed by cow's, goat's, or ass's milk or by artificial alimentation; but its prospect of life will be greatly, immeasurably, reduced. In addition to careful feeding a little careful tonic treatment should from the first be employed in conjunction with the specific remedies, iodide of iron, cod-liver oil, and preparations of the phosphates being the most useful drugs.

The existence of the second condition, which, as I have stated, exercises a modifying influence upon treatment—the early appearance of tertiary symptoms—is probably due in many cases at least to an overwhelming of the lymphatic system by the new cell-growth, which not only greatly increases the amount of material to be transported by the lymphatics, but at the same time, by invading their walls and diminishing their lumen, greatly cripples them. Accumulations of nutritive matter and of these new cells then take place, forming the characteristic new growths or deposits which we call gummata. This leads us to combine with the mercury from the beginning, at least in all cases where bony or periosteal involvement, suppuration, or the existence of gummata points to this condition, small doses of iodide of potassium or of some other soluble and easily decomposed iodine salt.

The principle of treatment being thus recognized, the routine procedure may be thus described: Give mercury as soon as the diagnosis of syphilis is assured—preferably by inunction. Sir Benjamin Brodie's opinion, expressed many years ago, still represents that of the profession:²¹⁵ "I have tried different ways of treating such cases. I have given the child gray powder internally and given mercury to the wet-nurse. But mercury exhibited to a child by the mouth generally gripes and purges, seldom doing any good, and given to the wet-nurse it does not answer very well, and certainly is a very cruel practice.²¹⁶ The mode in which I have treated cases for some years past has been this: I have spread mercurial ointment, made in the proportion of a drachm to an ounce, over a flannel roller and bound it around the child once a day. The child kicks about, and, the cuticle being thin, the mercury is absorbed. It does not either gripe or purge, nor does it make the gums sore, but it cures the disease. I have adopted this practice in a great many cases with signal success. Very few children recover in whom mercury is given internally, but I have not seen a case where this method of treatment has failed."

²¹⁵ Clinical Lectures on Surgery, Philada., 1846, p. 230.

²¹⁶ This, the so-called indirect method, is altogether unreliable, and should only be employed as a forlorn hope in those cases where in every other way mercury sets up gastro-intestinal irritation.

When, for any reason, as irritation of the skin, this cannot be employed, probably the best form of giving mercury by the mouth is in the following formula:

Rx.	Hydrarg. cum Creta,	gr. j to vj.
	Sacch. alb.	gr. xij.
In M. ft. chart No. xij.

S. One powder three times a day, to be taken soon after nursing.

Iodide of potassium may be given separately in a syrupy solution in doses of a half-grain to a grain, or if there are any marked tertiary symptoms even in much larger doses, three or four times daily.²¹⁷ Treatment should, of course, be continued long after the disappearance of syphilitic symptoms, and it would probably be well to continue the mixed treatment intermittently until after puberty.

²¹⁷ Wm. Campbell of Edinburgh was in the habit of commencing with doses of a quarter of a grain of calomel and two grains of creta præparata, once daily for the first ten days. He afterward progressively increased the calomel to a quarter of a grain twice each day. Sir John Rose Cormack says (Clinical Studies, vol. ii. pp. 423, 424, London, 1876) that an infant six weeks old will generally bear these doses well. In cases where they do not, he was in the habit of ordering a solution of half a grain of the bichloride in three ounces of distilled water and one ounce of syrup—one to two teaspoonfuls every six, eight, or twelve hours. When he used mercurial "swabbing" he employed from one to four drachms of unguent, hydrargyri to the ounce of lard. He alternated this treatment with short courses of the syrup of the iodide of iron, and continued the treatment up to the period of dentition. He says he has generally obtained excellent results by these methods.

With the treatment of special symptoms the general practitioner has little concern. The cases of visceral syphilis in very young children are generally fatal. Those that recover do so in response to the active use of the above remedies. Later, the prognosis is more favorable, the treatment the same. Of course moist eruptions should be dusted with some astringent or absorbent powder; mucous patches should be cauterized; and great attention should be paid to avoidance of sources of cutaneous irritation—frequent changing of diapers, etc.—but the general methods are the same as in the adult.