Definition And Description

The peculiarities of this affection are shown in the history of the following case.+

Johanna A., German, aged twenty-nine, came under my care December 20, 1870, with the following history: Two years and a half before, she discovered that the skin behind the left malleolus was hard and unyielding, like the calloused skin of a workingman's hand. This condition continued, and began to spread up the outer side of the leg in spite of various treatment. She occasionally suffered from pain in the affected limb, but the chief complaint was on account of the impaired mobility and strength of the member. There had been no appearance of the morbid condition upon any other part of the body. ++ The general health was good.

On examination, I found that the skin behind the malleolus was hard, white, and its outermost layers disposed to loosen and curl up; these, however, could not be removed without inflicting great pain. The skin was firmly bound down to the subjacent tissues and tightly stretched over the malleolus.

* I do not believe the quinine had anything to do with the return to health. + Published in the New York Medical Gazette, June 24, 1871. ++ Two or three years ago I again saw her. In the meantime the disease had appeared in much the same way on the right leg.

Above the ankle, the skin continued to present a sclerosed character, but appeared to be thicker than normal. As it was immovably attached, however, to the tissues beneath, its exact thickness could not be estimated. This condition extended to the upper third of the leg, and occupied the greater portion of its exterior and posterior aspects. The surface was somewhat heightened in color, and presented a slight tendency to exfoliation of the epidermis. Sensation tested by the aesthesiometer in-dicated equal tactility as compared with corresponding points upon the other leg, but there was increased sensitiveness to pain on the affected parts of the limb. The surface temperature of the sclerosed portion was much lower than on corresponding points of the right leg. Four and a half inches above the ankle, the left leg measured seven and a half inches in circumference, and the right leg eight inches.

The foregoing case exhibits strongly contrasting characteristics, as compared with the case of scleriasis previously given. In regard to scle-roderma generally, it may be stated that it occurs on almost every part of the body, having been observed on the neck, face, upper part of the neck, upper and lower extremities and elsewhere. It commences undoubtedly, though this has not been clearly determined, by a circumscribed infiltration of the skin and subcutaneous tissue.* The part affected is slightly elevated, and the skin of a brownish red color, with a very slight tendency to desquamation.

On touching the part it conveys to the finger a sensation of hardness, and the skin is found to be cemented to the subcutaneous tissue, and the whole tightly bound down to the muscles, or to the bones if they are near the surface. If we attempt to pinch up the skin into folds, the effort will be as futile as if we tried to pinch up the paint from a board. The surface temperature is lowered.

As the lesion gradually advances, the portions first affected undergo a change. The elevation subsides and gives place to depression, the heightened color disappears, and is replaced, first by a normal, later by a paler hue, and lastly by a glistening white. The tightness of the skin, its close adherence to the underlying tissues, and its absolute immobility become even more striking than in the early stage. The early hyperplasia is followed by aplasia. Sclerosed patches may appear upon several parts, and by gradual extension may involve a very large portion of the surface.

Diagnosis

The hide-bound condition above described occurs only in Scleroderma and Scleriasis. In the former of these, the affection commences by one or more limited patches, which very slowly increase in size, months or years elapsing before any great extent of surface is involved. In the latter the invasion is rapid, one to two weeks sufficing to implicate larger portions of the skin. In Scleroderma the parts affected ultimately become atrophied, if the patient does not sooner die. In Scleriasis, the skin is left intact when the disease passes off. In Scleroderma there is no tendency to spontaneous recovery. The reverse is the case in Scleriasis.

* I am not aware that any cases of ecleroderma hare been seen at the very incep-tion of the disease, and we are obliged to judge of the conditions then present, by observations at the advancing margin of the lesion.

In Scleroderma, treatment influences the affection favorably. In Scleria-sis I do not know that this is the case.

Prognosis

When limited in extent, Scleroderma does not appear to be specially prejudicial to life or health, but when extensive may be associated with visceral or other internal changes capable of inducing a fatal result.

Treatment

The treatment adopted in the case that came under my care in 1870, was reported in the Medical Gazette in 1871, as follows: Treatment was commenced on the 20th of December, by the application of the positive reophore of a constant current (galvanic battery) to the upper part of the left leg, and the negative was slowly moved over the affected parts. The applications were repeated on alternate days, and lasted from five to ten minutes at each sitting. Perceptible improvement was manifest after the fifth or sixth application. Improvement continued, and the patient was presented at the January and February (1871) meetings of the New York Dermatological Society. By the middle of March the skin above the malleolus had resumed its normal aspect, and was freely movable upon the cellular tissue. The portion which lay immediately over the malleolus was also freely movable over the bone, and its cicatricial appearance was somewhat modified, having lost, to some extent, its dead-white color. Considering the dermic condition substantially well, faradization of the muscles was commenced. This was continued for a few days only, as the patient felt herself so much recovered that she did not deem it worth while to pursue treatment further.* Since this case was reported, I have heard of several cases of Scleroderma treated in this country by means of electricity with varied success. More recently, Armaingaud + reports the successful use of galvanism in Scleroderma, and adds that "the result merits special mention, as the disease is usually rebellious to all medication, especially when of long standing, and the idea of applying electricity does not appear to have entered the minds of any one until now; not only have I failed to find mention of it in literature, but no reference to this method of treatment is made in the interesting clinical lectures of Prof. Hardy delivered at the Necker Hospital in 1877.

Hebra recommends tonic constitutional treatment.

Duhring (72, 364) recommends iron, arsenic, and cod-liver oil, together with the employment of baths and stimulating frictions in the form of liniments and ointments.