Anomalous Skin-Diseases

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

(Page 18 of 29)

Ichthyosis is a disease of the skin characterized by a morbid development of the papillae and thickening of the epidermic lamellae; according as the skin is affected over a larger or smaller area, or only the epithelial lining of the follicles, it is known as ichthyosis diffusa, or ichthyosis follicularis. The hardened masses of epithelium develop in excess, the epidermal layer loses in integrity, and the surface becomes scaled like that of a fish. Ichthyosis may be congenital, and over sixty years ago Steinhausen described a fetal monster in the anatomic collection in Berlin, the whole surface of whose body was covered with a thick layer of epidermis, the skin being so thick as to form a covering like a coat-of-mail. According to Rayer the celebrated "porcupine-man" who exhibited himself in England in 1710 was an example of a rare form of ichthyosis.

This man's body, except the face, the palms of the hands, and the soles of the feet, was covered with small excrescences in the form of prickles. These appendages were of a reddish-brown color, and so hard and elastic that they rustled and made a noise when the hand was passed over their surfaces. They appeared two months after birth and fell off every winter, to reappear each summer. In other respects the man was in very good health. He had six children, all of whom were covered with excrescences like himself. The hands of one of these children has been represented by Edwards in his "Gleanings of Natural History." A picture of the hand of the father is shown in the fifty-ninth volume of the Philosophical Transactions.

Pettigrew mentions a man with warty elongations encasing his whole body. At the parts where friction occurred the points of the elongations were worn off. This man was called "the biped armadillo." His great grandfather was found by a whaler in a wild state in Davis's Straits, and for four generations the male members of the family had been so encased. The females had normal skins. All the members of the well-known family of Lambert had the body covered with spines. Two members, brothers, aged twenty-two and fourteen, were examined by Geoffroy-Saint-Hilaire. This thickening of the epidermis and hair was the effect of some morbid predisposition which was transmitted from father to son, the daughters not being affected. Five generations could be reckoned which had been affected in the manner described.

The "porcupine-man" seen by Baker contracted small-pox, and his skin was temporarily freed from the squamae, but these reappeared shortly afterward. There are several older records of prickly men or porcupine-men. Ascanius mentions a porcupine-man, as do Buffon and Schreber. Autenreith speaks of a porcupine-man who was covered with innumerable verrucae. Martin described a remarkable variety of ichthyosis in which the skin was covered with strong hairs like the bristles of a boar. When numerous and thick the scales sometimes assumed a greenish-black hue. An example of this condition was the individual who exhibited under the name of the "alligator-boy." Figure 286 represents an "alligator-boy" exhibited by C. T. Taylor. The skin affected in this case resembled in color and consistency that of a young alligator. It was remarked that his olfactory sense was intact.

The harlequin fetus, of which there are specimens in Guy's Hospital, London Hospital, and the Royal College of Surgeons Museum, is the result of ichthyosis congenita. According to Crocker either after the removal of the vernix caseosa, which may be thick, or as the skin dries it is noticeably red, smooth, shiny, and in the more severe cases covered with actual plates. In the harlequin fetus the whole surface of the body is thickly covered with fatty epidermic plates, about 1/16 inch in thickness, which are broken up by horizontal and vertical fissures, and arranged transversely to the surface of the body like a loosely-built stone wall. After birth these fissures may extend down into the corium, and on movement produce much pain. The skin is so stiff and contracted that the eyes cannot be completely opened or shut, the lips are too stiff to permit of sucking, and are often inverted; the nose and ears are atrophied, the toes are contracted and cramped, and, if not born dead, the child soon dies from starvation and loss of heat. When the disease is less severe the child may survive some time. Crocker had a patient, a male child one month old, who survived three months. Hallopeau and Elliot also report similar cases.

Contagious follicular keratosis is an extremely rare affection in which there are peculiar, spine-like outgrowths, consisting in exudations of the mouths of the sebaceous glands. Leloir and Vidal shorten the name to acne cornee.

Erasmus Wilson speaks of it as ichthyosis sebacea cornea. H. G. Brooke describes a case in a girl of six. The first sign had been an eruption of little black spots on the nape of the neck. These spots gradually developed into papules, and the whole skin took on a dirty yellow color. Soon afterward the same appearances occurred on both shoulders, and, in the same order, spread gradually down the outer sides of the arms - first black specks, then papules, and lastly pigmentation. The black specks soon began to project, and comedo-like plugs and small, spine-like growths were produced. Both the spines and plugs were very hard and firmly-rooted. They resisted firm pressure with the forceps, and when placed on sheets of paper rattled like scraps of metal. A direct history of contagion was traced from this case to others.

Mibelli describes an uncommon form of keratodermia (porokeratosis). The patient was a man of twenty-one, and exhibited the following changes in his skin: On the left side of the neck, beyond a few centimeters below the lobe of the ear, there were about ten small warty patches, irregularly scattered, yellowish-brown in color, irregular in outline, and varying in size from a lentil to a half-franc piece, or rather more. Similar patches were seen on other portions of the face. Patches of varying size and form, sharply limited by a kind of small, peripheral "dike," sinuous but uninterrupted, of a color varying from red to whitish-red, dirty white, and to a hue but little different from that of the healthy skin. Similar patches were seen on the right hand, and again on the back of the right hand was a wide space, prolonged upward in the form of a broad band on the posterior surface of the forearm to just below the olecranon, where the skin was a little smoother and thinner than the surrounding skin, and altogether bare of hairs. The disease was noticed at the age of two, and gradually progressed. The patient always enjoyed the most perfect health, but had contracted syphilis three years before. A brother of the patient, aged twenty-four, for sixteen years has had the same skin-affection as this patient, on the back of the hand, and the sister and father had noticed similar lesions.