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Scleroderma

skin, affection, treatment, vessels, leading, hot and induration

SCLERODERMA Scleroderma IS Et chronic affection of the skin which usually com mences with oedematous swelling, leading to a coarse thickening and induration of the connective tissue of the skin and ending in atrophy.

According to the extent of the scleroderma we distinguish the universal forms (seE:reinies, Besnier) and the circumscribed forms (mor pheca). Generally speaking, three forms may be differentiated: (1) diffusely spread over the entire. body, commencing with coarse, glisten ing, (edematous swelling, gradually leading to a stone-hard induration, and often healing rapidly (sclCrodermie cedemateuse, Hardy); (2) the form eommencing symmetrically in the face, at the head and extremi ties, gradually becoming diffuse, often leading to sclerodactylia (isolated involvement of the hands with trophie disturbances of the muscles, vessels and bones) and designated sclerodermie progressive; (3) the localized forms which are sometimes called sclerodermic en plaques (rnorphoca, Wilson) and sometimes selerodermie en bandes, according to whether the eruption is arranged in roundish foci or in long stripes. In this form the freshly inflamed zone is frequently observed as a bluish ring, glistening with a violet tint, about 2 to 10 min. wide (lilac ring of the English), which encircles the coarsely infiltrated, atrophic yellowish or brownish centre.

The affection often commences with prodromal manifestations (sensation of cold, pain, itching etc.). There is also erythema or oedema at the attacked places, the skin becomes hard, rigid, difficult to lay- in folds, looks bluish white., arrests the movements, so that the face as sumes a peculiar masklike expression, while. the fingers acquire that immobility and rigidity which is known under the name of sclerodactylia. Gradually the affection leads to a thickening of the skin and atrophy, but it may also undergo spontaneous cure. Other signs are reduced sensibility and sweat secretion, falling cut of the hair, accompanied by increased pigmentation; facial lietniatrophy has been described by Neisser and Jagot.

The affection is found comparatively rarely in childhood, but may occur in the first few months of life (even in the second and third weeks—Cruse, Silbermann, Bahloni, Neumann). It attacks both boys and girls without distinction. II:lush:alter observed sclerodactylia in

a seven-year-old child.

Pathology.—The chief nature of the disease is an affection of the connective tissue which is changed to a swollen, homogeneous, glassy layer, appearing saturated with a viscous fluid which has coagulated (Neisser). The elastic fibres are reduced, the vessels are partly obliter ated, partly stenosed.

Etiology.—The cause of scleroderma is unknown. It is either a trophoneurosis or a nutritive disturbance of the connective tissue and vessels.

Prognosis.—The prognosis is relatively favorable in childhood, the affection running a lighter course than in adults; in the nursing period the affected places nearly always heal.

Diagnosis.—Recognition of scleroderma is comparatively easy frorn the induration, coarseness and rigidity of the skin.

Treatment.—Sternthal's dictum that "scleroderma either heals spontaneously- or not at all" is certainly not justified in view of the various methods of treatment by which the disease c'an bp favorably influenced. By internal treatment and invigorating diet the general condition should be fortified, and at the same time arsenic, quinine and strychnine administered, the best method being the three together in pills. Thyroidin has also been recommended. By local treatment the skin should be softened and the affected parts rendered more mobile. This is best accomplished by hot water and hot sand baths, hot douches, steaming, sweat cures and moist packs. The medicines to be most recom mended are salicylic preparations in connection with soap and salicylic soap plaster. In recent times experiments have been inade to soften tbe skin, which is as hard as a board, by the application of thiosinamin Mehra, Neisser, Galewsky). Every one or two days t to 1 c. c. of this substance with a 10 per cent. solution of glycerin is injected in the neighborhood of the diseased skin, or directly applied to the affected places in the shape of thiosinamin plasternaull. I have been very successful in this way in three cases. At the same time massage and electric treatment (constant current, or electric baths) are particularly valuable. Brocq recommends electrolysis for the treatment of the circumscribed forms.