LEPROSY.
BY JAMES C. WHITE, M.D.
DEFINITION.—Leprosy is a constitutional disease of chronic course and fatal termination, characterized by peculiar changes in the tissues of skin, mucous membrane, nerves, and most organs of the body.
SYNONYMS.—Elephantiasis of Greek writers; Lepra of Arabian authors; Anssatz (Germany); Spedalskhed (Norway). The local names in use among the numerous races in which it prevails are too numerous to be given here.
HISTORY.—Although great confusion has existed among the most ancient as well as later medical writers with regard to the definition of this disease, it having been confounded with several other affections (elephantiasis arabum, syphilis, psoriasis, morphoea, etc.), leprosy has prevailed in certain parts of the world from the time of the earliest records. The biblical accounts show that it existed among the Jews in Egypt, although it was not accurately distinguished from other diseases resembling it in some respects. It was recognized in Greece before the Christian era, and in the early centuries after Christ it had extended widely over Europe. In the seventh and eighth centuries special leper-houses were founded in Italy, France, and Germany. The disease reached its height in Europe in the twelfth and thirteenth centuries, when 19,000 lazarettos are said to have been in existence. Its spread was greatly increased by the constant intercourse kept up between Europe and the East during the Crusades. In the fifteenth century it began to diminish, and in the course of the seventeenth it had almost wholly disappeared from the most civilized states. It has lingered, however, in other parts, and exists to-day in France and Spain and Portugal, in Norway and Sweden, and in Italy, Greece, and Southern Russia. As in ancient times, it is widely spread along the coasts of Africa and prevails largely throughout Asia. It is found in many of the islands of the Indian and Pacific Oceans, in Japan, New Zealand, Madeira, the West Indies, extensively in some of the states of Central and South America and Mexico and the Hawaiian Islands.
It may be interesting to trace its history in the United States and adjacent districts more minutely. It is not known just when leprosy was introduced into North America. According to the Louisiana historian, Gayarré, the Spaniards established leper hospitals in several of their colonies on the Gulf of Mexico during the last century. One existed in New Orleans as late as 1785. In 1776 the disease was reported as existing among the blacks in Florida. It seems to have died out, and with it all remembrance of its former existence amongst us, until within the last few years, when its occurrence in the Southern States has again attracted attention. In Louisiana the first case was discovered in 1866 in an old woman whose father came from the south of France; she died in 1870. In 1871 it appeared in one of her sons, in 1872 in two others, and in 1876 in a nephew. A sixth case developed in a young woman who was in constant attendance upon the first case. In addition to this group, other cases have been observed in several parishes, amounting to twenty-one in all, as collected by Salomon of New Orleans in 1878.1 Two other cases, brother and sister, in Louisiana are known to the writer, one of whom has recently died under his care. In South Carolina the disease is reported by J. F. M. Geddings2 to have been observed in sixteen cases since the year 1846; four were Jews, four negroes, and eight whites. In none was any hereditary taint to be traced. No new cases have developed since that report.3
1 New Orleans Med. and Surg. Journal, March, 1878.
2 Trans. Intern. Med. Congress, Philadelphia, 1876.
3 See article on "Contagiousness of Leprosy" by writer, in Amer. Journ. of Med. Sciences, Oct., 1882.
In Minnesota and other North-western States leprosy has been known to exist for a considerable time among the Norwegian immigrants who have settled in them in large numbers. Holmboe in 1863 and Prof. Boeck later made visits to these colonies while in this country, and published reports concerning them after their return.4 The latter found eighteen cases among his countrymen, most of which were leprous before emigration; in others the disease developed after arrival in America. It had not manifested itself in any person born in this country. The character and progress of the affection seem to have been little influenced by residence here. Since these observations other cases have been collected by the committee on statistics of the American Dermatological Association,5 showing the continuance of the disease in these States. In 1879 there were fifteen cases in Minnesota. Its spread in this portion of our country is slow.
4 British and For. Med.-Chir. Review, Jan., 1870, and Nord. Medic. Ark., Bd. iii.
5 See Transactions.
Since 1871, 52 cases of the disease have been inmates of the hospital for lepers in San Francisco, California. Of these, all, with one exception, were Chinese, and forty-five of them had been sent back to China. It is presumed to have shown itself after arrival in this country, as "unproductive labor would not be imported by the Six Companies."6 No case of the disease known to have been acquired in this country has yet been reported upon the Pacific Coast. One case has developed in San Francisco after residence in the Hawaiian Islands.
6 Trans. Am. Derm. Assoc., 1881.
In Oregon, too, the disease has appeared among the Chinese immigrants, steps having been recently taken to re-ship five lepers from the poor-farm at Portland to China.
Since 1815, possibly earlier, leprosy has prevailed among the poor French settlements along the Miramichi River, near the Bay of Chaleurs, New Brunswick. It was first noticed in a woman whose mother came from Normandy, and has continued mainly in her descendants since. No measures were taken to control the disease until 1844, when a hospital was erected on Sheldrake Island. In 1849 the present lazaretto at Tracadie was established. During the first five years (1844-49) there were admitted 32 patients; from 1849 to 1863, 67 additional patients were received; and from the latter date to 1879, 30 more, making a total number of 129 up to the last report. The greatest number present at any one time was 37. In 1878 there were 16 patients in the lazaretto—6 men and 10 women. The total number of deaths in the hospital has been, up to 1878, 123. A. C. Smith, who resides near Tracadie, states that at the latter date but three cases were known to exist outside the lazaretto. Residence is not compulsory, and no sufficient measures are taken to remove patients from their homes before they may have inoculated other members of the family. The disease is more restricted in locality than formerly.
Within the last two years two or three small groups of the disease have been discovered in the island of Cape Breton, which are described in the Canadian Journal of Med. Science, Sept., 1881.
These are all the places north of Mexico where the disease exists in an endemic form. A considerable number of cases have been reported within the past few years from other parts of the United States, where it has manifested itself in persons who have formerly resided in leprous countries or in those who have wandered from the above infected districts. A very few instances have been recorded in which it has appeared in those who have never visited any infected locality or have been in apparent contact with lepers. Such cases, if authentic, establish the possibility of a sporadic origin of the affection. The fact of so many foci already established, and the penetration of a race so prone to the disease as the Chinese into all parts of the country, give the study of leprosy in America a special importance.
ETIOLOGY.—The study of the etiology of leprosy is intimately connected with that of its history and geographical distribution. From the earliest times it was regarded in all parts of the world as a contagious affection, and efforts were made by the sternest laws of Church and State to control its spread by segregation, by interdiction of marriage, etc. No disease has ever been regarded with an equal degree of abhorrence by mankind; none has received greater attention from physicians of every age. Within the present century it has come to be regarded, almost without exception, by the profession as non-contagious. Peculiarities of climate, soil, and modes of life have been looked upon as predisposing, exciting, or even essential influences in its causation; but the widespread distribution of the disease, with the consequent diversity of diet and customs of living, its prevalence upon the coast and in interior regions, in high altitudes as well as at the sea-level, in Iceland as in the tropics, show that these conditions, however they may affect the course of the affection, have no direct relation to its causation. The theory of heredity, as the most plausible explanation, has received its strongest support in the investigations of Boeck and Danielssen in Norway, where the disease can be traced for several generations in families. The same conclusions readily present themselves where the disease is studied in restricted localities, as in Louisiana and New Brunswick at the present time, where, as we have seen, it manifests itself closely in families in different generations. But this is a narrow point of view from which to study the etiology of leprosy. It often fails to manifest itself in the descendants of lepers in such communities, and affects persons in whose families it has never previously existed. Moreover, in countries where it does not prevail it not infrequently attacks individuals who have at some time visited regions where it was endemic, and in the latter places may develop in immigrants from parts of the world where it has never existed.
The same class of facts which seem to demonstrate its hereditary nature may be used in support of its infectious character. The proper field for observation in this regard would be a virgin region where its natural course could be studied independently of theories. Fortunately for science, such an opportunity is afforded in the history of the disease in the Hawaiian Islands. The exact date and mode of its introduction there are not definitely known. The islands have for years been the resort of the whaling-fleets manned by sailors coming from leprous regions. The natives also shipped as sailors, and after visiting such ports returned home. The absence of any restraint in the intercourse of crews and native women is well known. Isolated cases may have occurred as far back as 1830, but the disease made slow headway until about 1860, when it increased so rapidly that the government took stringent measures to control it, all cases discovered being sent to the leper segregation upon an island from which there is no escape. Since 1866, 2000 cases have been received there, and at last report the asylum contained 750 inmates. This by no means represents the extent of its prevalence in the islands, however. As the native population by recent census was only 44,000, it will be seen that the proportion affected is very large. This unwonted rapidity of spread cannot be accounted for on the ground of heredity. Transference from individual to individual by inoculation seems to be the only possible explanation, and all resident physicians believe that the disease is contagious in this sense. It affects almost exclusively those of native descent, and their habits of life are such as would greatly facilitate its wide dissemination in this way—viz. their great licentiousness and absence of all fear of the disease, which affords no bar to ordinary association or cohabitation; the crowding of large families in small huts and sharing the same mats and blankets; the eating of poi with the fingers from the same dish; passing a common drinking-vessel or pipe from mouth to mouth, etc.7 Promiscuous and compulsory vaccination with impure virus, too, has been generally practised during recent epidemics of small-pox. It is evident that abundant opportunity has in many ways been presented for the inoculation of pus or blood into the circulation from infected to healthy persons. Where immunity from contraction has followed marriage with a leper, it may be assumed that the conditions of an abraded surface and the contact with pus or blood have not been fulfilled. The wide spread of syphilis among the natives, and a consequent cachexia, have no doubt contributed to these conditions and established a national lack of resistance to the ravages of the disease. Nor can we overlook the proclivity of all endemic diseases to extraordinary manifestations of virulence in insular nations not previously protected by gradual inoculation. Many reliable cases are cited by resident physicians where the evidence of direct communication of the disease seems to be reliable. Facts of the same nature may be collected in the study of the history of the disease in New Brunswick and in Louisiana, where, as above stated, much better fields for investigating this question exist than in the Old-World regions where the affection has been rife for centuries.
7 Dr. G. W. Woods, U.S.N., in Hygienic and Med. Reports of Navy Department, vol. iv., 1879.
If we admit the fact of transference by inoculation in a single instance, there is no reason why we should not regard this as the principal if not the only means of extension of the disease, whether we accept or not the theory of its parasitic nature. It is not inconsistent with our knowledge of its laws and history to believe that leprosy is an affection communicated with difficulty, and after a prolonged period of incubation, from one person to another by contact with certain products of the diseased tissue; that it has in past and present time in this way spread from nation to nation; and that its progress as an endemic affection has been checked only by laws based upon this theory. All the negative facts so frequently urged against this doctrine of contagion apply as strongly to that of heredity, and may be interpreted in support of the former. The latest investigations into its pathology afford tangible evidence in its favor. It may at least be claimed that the question of contagion through inoculation must be reopened.8
8 See article on the question of contagion in leprosy in the American Journal of Med. Sciences, Oct., 1882, by the writer.
Leprosy affects both sexes in about equal degree, and may first show itself in early childhood. It is apt to produce sterility, so that marriages between lepers are rarely fruitful. This result seems to limit the extension of the disease under the law of heredity if we admit its action. There can be no doubt that cohabitation may take place for years without communication of the disease where one party alone is leprous; and such immunity may be explained by the failure of favorable conditions for sexual inoculation, just as in syphilis. The disease would naturally be most dangerous in its ulcerative tubercular form.
SYMPTOMATOLOGY.—There are two well-marked forms of leprosy—viz. the tubercular and the anæsthetic—which are characterized by certain easily recognized external manifestations, and which are accompanied by symptoms indicative of disturbances of the general economy as well as of special organs. These forms are not always sharply defined, and often occur simultaneously or in succession in individual cases. Both are generally preceded by premonitory symptoms, consisting of unaccountable languor of mind and body, tingling sensations in the skin, rise of temperature in the evening, and various disturbances of digestion, or by the occasional outbreak of single or several blebs. This prodromal stage affords no indication of the type of disease to follow, and may last for days, months, or even years, with greater or less intervals and intensity.
TUBERCULAR LEPROSY.—This form may declare itself at once by the characteristic tubercles, but frequently an earlier manifestation is the appearance of macules or dull red spots, varying in size from a pea to two or three inches in diameter. They have an indistinct margin, a glazed and smooth surface, and become paler on pressure. The patches, although not at all or but slightly elevated above the general surface, are firmer, and penetrate more or less deeply into the cutaneous tissues. They may increase in size peripherally and undergo involution in the older central portions simultaneously. During the latter process the color changes from a more or less dull red to a brown, yellow, or grayish tint, and finally may become quite white. The spots also become thinner or even slightly depressed. Their seat is principally the trunk, but also the limbs, and less frequently the face. This condition of the skin may precede any other changes in its tissues for months or years, the patches appearing and disappearing or remaining as permanent stains. At last well-defined tubercular elevations show themselves, varying in size from a small shot to a filbert, flattened or semi-globular in form, generally smooth and firm to the touch, and of a dull red or brown color. They occur upon any part of the surface, but are especially abundant upon the face, where they may cause great deformity of the features. The forehead and eyebrows may become very greatly thickened by general infiltration, or thrown out into very prominent folds and protuberances by the massing of individual tubercles. The lips thicken, the nose broadens, and the ears stand out conspicuously with their increased bulk. All these changes in form, with the great darkening in tint which is often present, give at times a most repulsive expression to the face. The tubercles are sometimes to be felt imbedded in the skin, or considerable areas are found to be uniformly thickened and scarcely at all prominent. All forms are capable of involution after an existence of months, and may leave dark-colored atrophic patches to mark their seat. They are rarely painful, and occasionally slightly sensitive. They may be transformed into ulcers, especially upon prominent positions, as the knuckles, elbows, knees, as the result of pressure or injury, which are extremely indolent, although shallow, and may heal and break down repeatedly. Occasionally they give rise to serious complications—inflammation of the lymph-vessels, suppuration of the joints with loss of the attendant members, as the fingers and toes. Tubercles appear also upon the mucous membrane of the nasal cavities, the mouth, and larynx, often in great abundance, causing a very characteristic hoarseness or loss of voice. With these changes in the cutaneous tissues, which may be accompanied in their periods of greatest activity by febrile disturbances, there are developed after months or years, with gradual failure of strength, manifestations of changes in the internal organs, the lungs, intestines, and brain, which may prove fatal at any time, or the patient may die of slowly progressive marasmus. The course of the tubercular form is on the average between eight and ten years. At any period there may supervene manifestations of the anæsthetic type, which makes the so-called mixed variety, in which either form may predominate.
ANÆSTHETIC LEPROSY.—This variety is characterized by the loss of sensation in the skin over areas of varying extent, which occupy no definite positions in relation to nerve-distribution. The anæsthetic patches may appear upon the seat of old maculæ or former tubercles or of a preceding bullous efflorescence, or upon parts not previously affected in any way. They may follow a reddened and hyperæsthetic condition of the cutaneous tissues, or they may be surrounded by a serpiginous border of this character. The degree of anæsthesia in the affected parts is sometimes so complete that the skin and underlying tissues may be deeply pricked or cut or burned without the patient being aware of the injury. Such patches may possibly regain their sensibility. Their surface appears in later stages dry, wrinkled, shrunken, and of a brownish color, and atrophy, not only of the skin but of the muscles, is gradually developed, in consequence of which the expression of the face undergoes a marked change. The eyelids and lips droop, the hair falls, the hands contract, and the joints of the fingers and toes are laid bare, so that the phalanges, or even the whole hands and feet, drop off. Ulceration or gangrene of the parts may develop, and whole extremities may shrivel up. With these manifestations of local derangements of nerve-action the functions of the brain fail, the patient becoming stupid and incapable of action or motion, the temperature and pulse are lowered, and death comes slowly by marasmus or the most various complications—tetanus, disease of the lungs, pyæmia, etc. The average duration of this form is from eighteen to twenty years.
PATHOLOGICAL ANATOMY.—The structural changes which take place in the tissues of parts which are the seat of the appearances above described have received the special study of many excellent observers9 in recent times, and are now well understood. A section through the thickened skin or a tubercle shows the corium and underlying connective tissue infiltrated with round cells, as in lupus and syphilis; in other words, converted into "granulation tissue." This change first takes place along the course of the cutaneous vessels and glands, penetrating more deeply and forming a firmer cell new-growth in proportion to duration, the cells being enclosed in a coarse meshwork of fibrous tissue, and encroaching upon the various structures of the skin, so as to produce atrophy and finally destruction of all its characteristic tissues. This cell-infiltration may of itself undergo later changes, as fatty degeneration and softening (ulceration). The lymph-glands and corpuscles assume a special fatty metamorphosis. An examination of the tubercles upon the mucous membrane reveals the same small-celled new-growth. In the nerve-tissues also marked structural changes are found, both in the central and peripheral systems, in the anæsthetic form of the disease. In many cases the posterior segments of the gray cornua and the fibres of the commissure, as well as the nerves of the extremities, have been found altered by inflammation, which will account for the disordered sensibility and the subsequent disturbances of nutrition, muscular atrophy, etc. The nerve-trunks are often to be felt beneath the skin, thickened and sensitive on pressure. The chronic cell-infiltration affects the fibrous structure of the outer sheath, the neurilemma, and the septa between the nerve-bundles, producing fatty metamorphosis and atrophy of the nerve-bundles. Similar cell-infiltrations are found also in the connective tissue of all the internal organs of the body, which lead to destructive processes in their respective structures.
9 Boeck and Danielssen, Traité de la Spedalskhed, Paris, 1848; Virchow, Die Krankhaften Geschwülste; Kaposi in Hebra's Lehrbuch der Hautkrankheiten; Monasterski, Vierteljahressch. für Derm. u. Syph., 1879, p. 203; Hansen, Virchow's Archiv, Band 79, 1880; Neisser, Virchow's Archiv, Band 84, 1881; Cornil et Souchard, Annales de Derm. et de Syph., 1881, No. 4.
Within the last two years repeated observations have been made which confirm the statement published by Hansen in 1873, that a peculiar bacterium occurs in leprous tissues, which, it is claimed, establishes the parasitic nature of the affection. These examinations have been carried on with leprous material derived from many parts of the world, and the results have been uniform. Within the round cells which characterize the cutaneous neoplasms, both in the distinct tubercles and the diffused infiltrations, small agglomerations of minute rod- or staff-like bodies (bacilli) are found, arranged in parallel rows or placed end to end. Their length is one-half or three-fourths the diameter of a red blood-globule, and their breadth is one-fourth their length. With them minute granular particles are seen in the cells. They occur in greatest numbers in the cells of the upper layers of the true skin, which are considerably swollen by their presence. They never penetrate the epithelial layer, nor are they found in epithelial cells in any position. When the protoplasm of the cell is interfered with by the later tissue-changes of the disease, the bacillus perishes. They are found not only in the leprous cells, but also in those of the connective tissue running between the agglomerated masses of the former. Between the leprous cells and the filaments of connective tissue but few free bacilli are seen. The neoplasms of the mucous membrane and of many organs of the body have been found to contain them also. In the blood they have been detected by some observers. Their presence in the nerve-tissues is of importance as throwing light upon the question of the specific or inflammatory nature of the morbid processes above described as affecting them. If we regard the bacteria as pathognomonic of leprous tissue-changes, their occurrence, recognized in the cells penetrating between the fibres of the peripheral nerves, would seem to make all primary structural changes identical, and the anæsthetic as much as the tubercular form the direct result of their presence. Neisser draws the following conclusions from his investigations: "Leprosy is a real bacterial disease, caused by a special kind of bacterium. The bacilli appear in the tissues as such, or more probably as spores, and remain for a longer or shorter time in a state of incubation, according to circumstances, in dépôts, perhaps in the lymph-glands. This period, much longer than in other infective diseases, is in proportion to the physiological resistance of the human organism compared with the feeble developing power of the bacilli. It, as well as the course of the disease, is more rapid in tropical countries than in Europe. From these dépôts the disease extends throughout the body in those portions of the skin most exposed, the face, hands, elbows, knees, and into the peripheral nerves. The other organs are less freely invaded. The bacilli excite inflammation, and by a specific action transform the migrating cell into the leprous cell. Leprosy is probably an infectious disease, and its specific products are contagious—viz. the leprous cells of the tubercles, the tissue-fluids, and the pus containing bacilli or viable spores. On the other hand, the pus may not always be infectious, as the fluid contained in the bullæ is not."
It must be said that the bacterial nature of leprosy, if established in accordance with the above observations, furnishes a satisfactory basis of explanation of all facts, historical, clinical, and pathological, which have so long been awaiting solution. The inability of the parasite to penetrate the epithelial layer of the skin and mucous membrane explains why contagion is so difficult, and why the ulcerative tubercular form would be more favorable to such transference than the anæsthetic variety.
DIAGNOSIS.—Leprosy in some of its early appearances may be readily confounded with vitiligo, morphoea, pemphigus, lupus, and syphilis. In some cases its prodromal manifestations cannot be positively diagnosticated until other symptoms have developed, which by concurrence establish their true significance. Such are the pemphigus-like bullæ, the pigment-changes, and the smaller tubercular efflorescences. In regions where the disease occurs only by importation, and in the so-called sporadic cases, it is not at all strange that it should fail of recognition, even in well-advanced forms, unless the observer is acquainted with its whole symptomatology. On the other hand, there is no disease which presents more strikingly characteristic features in its advanced stages.
PROGNOSIS.—Leprosy is almost uniformly a fatal affection, and its course toward this termination varies but slightly under the most diverse conditions of life. Its development and progress are naturally more rapid under circumstances of least individual resistance, where food is poor and scanty, where extremes of climate are most felt, where the constitution of the individual or nation is debilitated by previous disease, as that of the Hawaiians by syphilis, or where no proper professional care is employed. It has been believed that a change of residence from infected to non-leprous regions would retard its advance or avert its appearance in those supposed to be hereditarily disposed; but the former effect follows probably only so far as the general condition of the patient is affected by the change, as in other constitutional disorders, and the latter is necessarily a matter wholly of conjecture. No case of leprosy in the Norwegian colony in our North-western States has ceased to progress after arrival toward its fatal ending, even if this has been somewhat delayed in individual cases under more generous ways of living. If it could be known that a child born in Norway had escaped leprosy by removal to America, we should not, if we accept the bacterial origin of the disease, consider that climate or other mysterious influences had overcome its inherited tendencies, but that it had been taken away from the chance of direct inoculation. It is stated that very rarely cases cease to progress beyond certain stages even in countries where the disease is endemic. The course, as has been stated, varies according to the clinical form, the duration of the tubercular variety being on an average but one-half that of the purely anæsthetic type. Leprosy may be called the slow disease, its period of incubation, so far as this can be determined, extending from one to several years, its prodromal stage lasting often several more years, and its well-developed forms requiring at times more than twenty years to destroy the patient. Cases sometimes prove fatal, however, in a single year.
TREATMENT.—In a disease which affects so many of the races and such great numbers of mankind, which has been for centuries the object of special attention on the part of physicians, and of late years of government commissions and of eminent pathologists, it is evident that every remedy which the materia medica includes, as well as those of merely popular reputation in the widely-diverse geographical regions in which it prevails, must have been employed in its treatment. None of them exert any specific action upon it; it remains incurable. Every year some new article is employed with the usual claims of success which accompany the introduction of new remedies, but they merely swell the long list of failures in the therapeutics of the affection. Still, leprosy is influenced somewhat by medical care; life may be prolonged and made more comfortable. To this end we may employ remedies which are capable of improving and maintaining the constitutional powers of resistance to the disease, such as are found of service in other chronic wasting affections. The patient is to be put in as healthy ways of living as possible, removed from debilitating localities, and given generous diet and tonics, as iron and quinia. Several new drugs which seem to stimulate the nutrition and produce temporary improvement in the local and general symptoms have lately been widely employed, as Gurjun balsam and chaulmoogra oil, but they have wrought no cure. Digestion is to be aided, diarrhoea to be checked, and disturbances of respiration to be alleviated. Local treatment is also of service. The tubercles may sometimes be made to disappear—partly, at least—by stimulating applications, and ulcers made to heal by cauterization and other well-known methods of dressing. These ulcers and their secretions should be regarded as possible sources of infection by attendants and members of the patient's household. For the anæsthetic alterations in the tissues but little can be done locally. If the bacterial origin and causation of the disease be eventually established, its future extinction must be based upon studies directed to the nature and mode of protection against this organism. Collectively, the disease should be treated by every nation by thorough segregation, and importation should be prevented by the most rigid quarantine laws.