SCLERODERMA.
Definition. — A disease characterized by induration of the skin, and at times of the subcutaneous tissues, which some times progresses to complete atrophy of these tissues.
Varieties. — Three main varieties of scleroderma are recognized: the diffuse, which is generalized or limited to certain areas; the circumscribed, or morphea, Nvhich appears in spots; and sclerodac lyly, which is limited to the hands.
Symptoms.—In the diffuse form, after a series of prodromic symptoms, sensa tions of chilliness or heat, pruritus, and pain in the muscles and articulations, the tissues become thickened, stiff, and hard, and appear (edematous. The skin is cold and whitish, contracted, and sometimes painful. The face and the upper part of the body may be the only partS af fected, but the entire body becomes in volved. The skin is, as it were, glued to the skeleton, the fingers and toes be ing thin and stiff or hooked. Gangrene is sometimes observed, constituting the mutilating form. The prognosis is ex ceedingly unfavorable as regards cure.
In the circumscribed variety, the mor phea of Erasmus Wilson, the affected spots are limited in area, the spots being flat or raised, oval or rounded. Their color varies from a light pink to a pale or dark violet, and undergoes changes which ultimately give the lesion a characteristic aspect: a whitish-brown squamous centre surrounded by a bluish or lilac pigmented border, or ring. They are seldom painful, though pruritus is sometimes complained of. The spots, of which there are generally but two or three, are usually located upon the neck, the chest, the abdomen, the arms, or the thighs. These spots gradually fade away, but, occasionally, cicatrices are left to mark the location of the lesions. The prognosis in this form is favorable.
In sclerodactyly the third phalanx be comes atrophied and its tissues, includ ing the nail, are partially destroyed by abscess. The flexor tendons are con tracted and give the finger the appear ance of an angular hook by flexing the first phalanx upon the second. Here also the skin is hard, contracted, adherent to the bones, and lilac in color. The prog nosis is necessarily unfavorable, owing to the mutilations caused by the disease.
Diagnosis. — The only condition with which scleroderma can be easily con founded is leprosy, but the tubercles of the latter disease, the broad dissemina tion of the skin-lesions, the nasal dis order, the character of the ulcerations, and the disturbances of sensation usu ally facilitate its recognition.
Diffuse scleroderma must sometimes be distinguished from brawny, solid oedema, met with at times in patients with long standing renal or cardiac disease, in which there is induration following chronic dropsy. In scorbutic sclerosis
there may be parchment-like immobility of the skin, due to extensive subcutane ous hemorrhages, involving the muscles. In the stage of swelling it may resemble myxcedema. In Raynaud's disease the infiltration, pigmentation, and extreme cyanosis are not wholly unlike those of scleroderma. The increase of pigment may suggest Addison's disease, since the bronzing may be extreme. Osler (Jour. Cutan. and Genito-Urin. Dis., Feb., Mar., '9S).
Case of diffuse scleroderma in a man of 41 years with total atrophy of the thyroid gland and bronzing of the skin. The scleroderma affected the hands, the face, the chest, the abdomen, and the lower extremities. Sensation, both for temperature and pain, was normal; the electrical reactions were practically un changed; the s.veat-secretion was not disturbed. Atrophy of the thyroid gland has been found in other cases of sclero derma, but it was generally secondary; in this case it seemed to precede the skin-changes. Uhlenhuth (Berl. klin. Woch., Mar. '99).
Etiology and Pathology.—Scleroderma is a trophoneurosis, most frequently ob served among neurotic subjects and often in connection with the rheumatic di athesis. It may appear at any age, but is more prevalent among women than men. The neurotic influence, however, , does not account for all cases, nerve- ' changes being wanting in the majority.
liaposi notes that the lesions follow, to a degree, vascular distribution. The morbid changes peculiar to scleroderma include an end operiarteritis, which may be traced to various structures, the mus cles, the myocardium, the uterus, the lungs, and the kidneys particularly. The sclerosis would thus seem to be a result of the vascular disturbances, through impaired nutrition of the affected areas.
Treatment. — The treatment consists in nutritious diet, iron, and codliver-oil in ascending doses (the latter up to 10 tablespoonfuls per day); sodium salic ylate; externally, steam-baths, mud baths, mercury, galvanism, and massage. The most recent remedy is thyroid gland; but, according to Osler, it is not of much value. Brocq recommends electrolysis, at first at comparatively short intervals; then, when amelioration is manifest, at much longer intervals. Electrolysis does not act by destructive action, but at a distance, influencing even patches not touched. Philippsohn obtained excellent results by the administration of salol, in doses of about 7 to 15 grains, three or four times daily.