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.
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. Adisease 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 98degrees F. In a little more than four months the skin became normal. The treatment with mercurial ointment was stopped some time before recovery.
