CHAPTER IV
PATHOLOGY
Physicians have had a general knowledge of the pathology of scurvy for a great many years. Lind, in his “Treatise on the Scurvy,” published in 1772, included a chapter on “dissections” and a postscript on “Appearances on Dissections of Scorbutic Bodies,” based on a large, although indefinite, number of postmortem examinations. In the century which followed, there are to be found many reports of scurvy, especially in connection with the frequent wars, but it is surprising how little detailed pathologic information they furnish. Barlow’s publication in 1883, establishing the identity of the scurvy of adults and of infants, must be regarded as the modern milestone in the study of the pathology of this disorder. This work did not contribute richly to the data of the subject, or suggest novel interpretations, but directed attention to a new source of material—the increasing number of cases of infantile scurvy—at a critical moment when the opportunity for the study of scurvy in the adult was rapidly becoming less. At the time of Barlow’s exposition of the true nature of “acute rickets,” scientific medicine was concentrating its interest on pathology. Tissues were being carefully studied by means of the microscope, and scurvy was subjected to this new method of investigation. As a result of intensive application of this technic, a lesion of the bones was identified and established as characteristic of scurvy. Study was focussed so exclusively on the bones, that for many years, indeed until very recently, the other organs of the body were neglected. This is true of the gross as well as of the microscopic anatomy. Protocol after protocol gives a hasty account of the appearance of the various organs, merely as a routine introduction to a careful and often minute study of the bones (Table 2). As the result of this myopic vision, enlargement of the heart, for example, which should have been noted many years ago, was, until recently, unobserved—indeed, the heart is but occasionally mentioned in the protocols.
| Number of Cases | Author | Date | Gross | Microscopic |
|---|---|---|---|---|
| Adults | ||||
| Many | Lind | 1772 | Brief summaries | |
| 51 | von Himmelstein | 1843 | Brief summaries | |
| 8 | Hayem | 1871 | Résumé | Brief résumé. |
| 7 | Lasèque & Legroux | 1871 | Fairly detailed | |
| 7 | Charpentier | 1871 | Brief summaries | |
| 13 | Sato & Nambu | 1908 | Fairly detailed | Fairly detailed. |
| 2 | Urizio | 1917 | Brief summaries | Brief summaries. |
| 1 | Feigenbaum | 1917 | Brief summary | Brief summary. |
| 23 | Aschoff & Koch | 1919 | Very complete | Very complete. |
| Infants and Children | ||||
| 1 | Smith | 1876 | Brief report | |
| 3 | Barlow | 1883 | Fairly detailed | Bones, brief report. |
| 1 | MacKenzie | 1883 | Brief summary | |
| 1 | Northrup | 1892 | Brief report | |
| 1 | Cassel | 1893 | Brief report | |
| 2 | Sutherland | 1894 | Fairly detailed | Brain and liver, 1 case. |
| 1 | Reinert | 1895 | Very complete | Fairly complete. |
| 1 | Hirschsprung | 1896 | Summary | |
| 3 | Meyer | 1896 | Fairly detailed | |
| 1 | Baginsky | 1897 | Fairly detailed | Brief, bones more in detail. |
| 1 | Naegeli | 1897 | Fairly detailed | Bones only. |
| 1 | Manz | 1899 | Summary | |
| 5 | Schoedel & Nauwerk | 1900 | Very complete | Very complete. |
| 1 | Jacobsthal | 1900 | Very complete | Very complete. |
| 1 | Stoos | 1903 | Fairly complete | Bones only. |
| 1 | Looser | 1905 | Summary | |
| 1 | Hoffmann | 1905 | Summary | Bones only. |
| 1 | Stoeltzner | 1906 | Bone only | Bone only. |
| 22 | Fraenkel | 1908 | Résumé | Bones only. |
| 1 | Nobécourt et al | 1913 | Brief report | Bone only. |
| 3 | Ingier | 1913 | Brief report | Bones, very complete. |
| 1 | Bahrdt & Edelstein | 1913 | Complete | Bones, brief report. |
| 31 | Erdheim | 1918 | Brief summaries | Brief résumé. |
| 1 | Epstein | 1918 | Very detailed | |
A new era in the pathology of scurvy was inaugurated by the availability of experimental scurvy and also by the stimulation occasioned by the recent conception of vitamines and the so-called deficiency diseases. In endeavoring to elucidate this fascinating problem, it has gradually been realized that pathology may be of service—for example, in relation to the involvement of the endocrine glands. Accordingly, studies of the minute pathology of the various organs have been undertaken in many laboratories throughout the world (Italy, India, England, Germany and the United States). An additional stimulus to investigation in pathology has been furnished by the recent war, which, as shown elsewhere, led to a great increase in scurvy among both the military and civilian population. The excellent report of Aschoff and Koch from Roumania was made possible by this catastrophe, and will no doubt soon be followed by others of similar character.
Gross Pathology.—General Appearance.—The skin usually is pale, livid, and dotted with numerous petechiæ. These vary in size from the tiniest pin-points, barely recognizable to the naked eye, to ecchymoses of moderately large size. The most frequent site is the lower extremities. The trunk is always less affected, hemorrhages tending to occur along the mid-line and especially around the umbilicus. There may be also larger superficial hemorrhages, showing great differences in color, from the redder tone of the more recent, to the blues, browns and greens of the older lesions. Bleeding from the nose and mouth is not uncommon in fatal scurvy, and occasionally exophthalmos is present, usually unilateral, and due to subperiosteal hemorrhage of the orbital plate of the frontal bone. Rigor mortis is generally slight, and, according to Lind and to von Opitz, decomposition takes place rapidly.
There may be great emaciation, especially where secondary infection has supervened. General wasting occurs, however, in uncomplicated scurvy due to starvation—the result of lack of appetite or a deficiency of the general food supply. Children, especially infants, are undersized, as illustrated in treating of the symptomatology, and their bones may be decidedly smaller than normal. Generally there is some edema about the ankles, and in children a somewhat characteristic puffiness about the eyes. General anasarca also occurs, in some cases associated with renal involvement. Peculiar boggy, “tumor-like” masses of localized edema may be present, which were considered by the earlier writers (Lind) to be one of the typical lesions of this condition.
Hemorrhages.—Hemorrhage is such a striking manifestation that it is not surprising to find it was regarded by the older writers as the pathognomonic sign of scurvy. The bleeding may take place into almost any organ, and vary from small petechiæ to very extensive extravasations. The hair follicles and sweat glands are particularly susceptible, as Lasèque and Legroux noticed in cases occurring in the French prisons. Aschoff and Koch, during the recent war, noted the same peculiarity of involvement, calling attention also to the fact that previous skin diseases such as keratosis or seborrhea seemed to predispose to this localization.
Trauma plays a very important rôle in determining the location of the deeper as well as of the superficial hemorrhages. In adults, especially in soldiers, in whom the greater number of cases have been recorded, the lower extremity is the commonest site, between the knee and ankle, the area most exposed to blows as well as acted upon by static congestion. In infants, the inner aspect of the thighs is a frequent site due to the trauma of the diaper.
The most characteristic hemorrhage, the subperiosteal, will be fully discussed when considering the bone lesions. The deeper hemorrhages may be very extensive. They tend to follow the connective-tissue strata, and in the muscles are usually limited by the muscle sheaths. The blood surrounds the muscle fibres, which appear quite intact. The neighboring blood-vessels are congested and may contain thrombi, both venous and arterial. Such thrombi are found also in areas where extravasation has not taken place, and conversely, hemorrhages occur where no thrombi are demonstrable, so that a mutual causal relationship cannot be proved. Further evidence in regard to the mechanism of these extravasations is presented in the discussion of the minute anatomy. Brownish pigment, undoubtedly derived from the blood, is frequently found in the neighborhood of the hemorrhagic areas. New connective tissue also grows in these areas, so that in healing cases a marked formation of scar tissue will be found. Bizarre forms of hemorrhage at times have given rise to confusion in diagnosis; hemorrhage involving the right lower abdominal quadrant may simulate appendicitis; when occurring in the region of the transverse colon it has been mistaken for an epigastric new-growth.