Anomalous Skin-Diseases, Part 2

Anomalies and Curiosities of Medicine
by George M. Gould, M.D., Walter L. Pyle, M.D.

(Page 19 of 29)

Diffuse symmetric scleroderma, or hide-bound disease, is quite rare, and presents itself in two phases: that of infiltration (more properly called hypertrophy) and atrophy, caused by shrinkage. The whole body may be involved, and each joint may be fixed as the skin over it becomes rigid. The muscles may be implicated independently of the skin, or simultaneously, and they give the resemblance of rigor mortis. The whole skin is so hard as to suggest the idea of a frozen corpse, without the coldness, the temperature being only slightly subnormal. The skin can neither be pitted nor pinched. As Crocker has well put it, when the face is affected it is gorgonized, so to speak, both to the eye and to the touch. The mouth cannot be opened; the lids usually escape, but if involved they are half closed, and in either case immovable.

The effect of the disease on the chest-walls is to seriously interfere with the respiration and to flatten and almost obliterate the breasts; as to the limbs, from the shortening of the distended skin the joints are fixed in a more or less rigid position. The mucous membranes may be affected, and the secretion of both sweat and sebum is diminished in proportion to the degree of the affection, and may be quite absent. The atrophic type of scleroderma is preceded by an edema, and from pressure-atrophy of the fat and muscles the skin of the face is strained over the bones; the lips are shortened, the gums shrink from the teeth and lead to caries, and the nostrils are compressed. The strained skin and the emotionless features (relieved only by telangiectatic striae) give the countenance a ghastly, corpse-like aspect. The etiology and pathology of this disease are quite obscure. Happily the prognosis is good, as there is a tendency to spontaneous recovery, although the convalescence may be extended.

Although regarded by many as a disease distinct from scleroderma, morphea is best described as a circumscribed scleroderma, and presents itself in two clinical aspects: patches and bands, the patches being the more common.

Scleroderma neonatorum is an induration of the skin, congenital and occurring soon after birth, and is invariably fatal. A disease somewhat analogous is edema neonatorum, which is a subcutaneous edema with induration affecting the new-born. If complete it is invariably fatal, but in a few cases in which the process has been incomplete recovery has occurred. Gerard reports recovery from a case of sclerema neonatorum in an infant five weeks old, which seemed in perfect health but for this skin-affection. The back presented a remarkable induration which involved the entire dorsal aspect, including the deltoid regions, the upper arms, the buttocks, and the thighs, down to and involving the popliteal spaces. The edges of the indurated skin were sharply defined, irregular, and map-like. The affected skin was stretched, but not shiny, and exhibited a pink mottling; it could not be pinched between the fingers; pressure produced no pitting, but rendered the surface pale for a time. The induration upon the buttocks had been noticed immediately after birth, and the region was at first of a deep pink color. During the first nine days the trouble had extended to the thighs, but only shortly before the examination had it attacked the arms. Inunctions of codliver oil were at first used, but with little improvement. Blue ointment was substituted, and improvement commenced. As the induration cleared up, outlying patches of the affected skin were left surrounded by normal integument. No pitting could be produced even after the tension of the skin had decreased during recovery. The lowest rectal temperature was 98 degrees F. In a little more than four months the skin became normal. The treatment with mercurial ointment was stopped some time before recovery.

Possibly the most interesting of the examples of skin-anomaly was the "elephant-man" of London. His real name was Merrick. He was born at Leicester, and gave an elaborate account of shock experienced by his mother shortly before his birth, when she was knocked down by an elephant at a circus; to this circumstance he attributed his unfortunate condition. He derived his name from a proboscis-like projection of his nose and lips, together with a peculiar deformity of the forehead. He was victimized by showmen during his early life, and for a time was shown in Whitechapel Road, where his exhibition was stopped by the police. He was afterward shown in Belgium, and was there plundered of all his savings. The gruesome spectacle he presented ostracized him from the pleasures of friendship and society, and sometimes interfered with his travels. On one occasion a steamboat captain refused to take him as a passenger. Treves exhibited him twice before the Pathological Society of London.

His affection was not elephantiasis, but a complication of congenital hypertrophy of certain bones and pachydermatocele and papilloma of the skin. From his youth he suffered from a disease of the left hip-joint. The papillary masses developed on the skin of the back, buttock, and occiput. In the right pectoral and posterior aspect of the right axillary region, and over the buttocks, the affected skin hung in heavy pendulous flaps. His left arm was free from disease. His head grew so heavy that at length he had great difficulty in holding it up. He slept in a sitting or crouching position, with his hands clasped over his legs, and his head on his knees. If he lay down flat, the heavy head showed a tendency to fall back and produce a sense of suffocation. For a long time he was an inmate of the London Hospital, where special quarters were provided for him, and it was there that he was found dead, April 11, 1890; while in bed his ponderous head had fallen backward and dislocated his neck.

Ainhum may be defined as a pathologic process, the ultimate result of which is a spontaneous amputation of the little toe. It is confined almost exclusively to negroes, chiefly males, and of African descent. In Brazil it is called "ainham" or "quigila." "Ainham" literally means to saw, and is doubtless a colloquial name derived from a supposed slow, sawing process. The Hindoo name for it is "sukha pakla," meaning dry suppuration.

In 1866 da Silva Lima of Bahia, at the Misericordia Hospital, gave the first reports of this curious disease, and for quite a period it was supposed to be confined to Brazilian territory. Since then, however, it has been reported from nearly every quarter of the globe. Relative to its geographic distribution, Pyle states that da Silva Lima and Seixas of Bahia have reported numerous cases in Brazil, as have Figueredo, Pereira, Pirovano, Alpin, and Guimares. Toppin reports it in Pernambuco. Mr. Milton reports a case from Cairo, and Dr. Creswell at Suez, both in slaves. E. A. G. Doyle reports several cases at the Fernando Hospital, Trinidad. Digby reports its prevalence on the west coast of Africa, particularly among a race of negroes called Krumens. Messum reports it in the South African Republic, and speaks of its prevalence among the Kaffirs.