SCLERODERMA

Synonyms: Dermatosclerosis; Sclerema; Scleroma; Scleriasis; Hide bound disease.

The most characteristic feature of scleroderma is, as the name indicates, a hardening of the skin, and this may be the only feature in common between the two most widely separated varieties. The lesions may develop rapidly, or as is more common, very gradually; they may be single or multiple, diffuse and ill defined or localized and sharply circumscribed, level with the normal skin or slightly elevated or depressed, of an ivory like whiteness or a translucent yellow, or pigmented diffusely or in blotches. The patches sometimes have a characteristic violaceous areola. Their surface is usually smooth but may be slightly scaly or somewhat nodular and is often traversed by a network of dilated capillaries. The evolution of the patches may be quite insidious or preceded or accompanied by moderate burning pain or pruritus. Neighboring plaques may coalesce and sometimes enclose islands of normal skin. In all cases the integument feels thick and is often leathery and so bound down to the deeper structures that it cannot be pinched up in folds. In the diffuse form the progressive thickening and shrinking of the skin may greatly interfere with the function and nutrition of the parts beneath. On a limb the muscles may atrophy and the joints become ankylosed. In the condition termed sclerodactylia the hands and fingers are rendered stiff, immobile and useless. When the integument

of the chest is involved respiration may be greatly interfered with. On the face the natural folds disappear, movements of the mouth and eyelids are much inhibited and the face assumes an expressionless and cadaveric appearance. When the condition is very extensive it usually causes marasmus and death. In some cases after months or years the infiltration disappears gradually and leaves the skin thin, dry, wrinkled and parchment like. The more common circumscribed variety, also known as *morphoea*, is usually slower in its development and more prone to recover in time and leave either a scar like atrophy or merely depressions caused by loss of subcutaneous structures, or no traces at all. New patches may develop while others disappear. Sometimes the disease limits itself chiefly to one side of the face and produces a more or less marked facial hemiatrophy. The course of scleroderma is variable, there may be periods of improvement and recrudescence and the disease may become arrested at any stage. The etiology is obscure. The disease occurs three times as frequently in women as in men and is most common in youth and middle age. **TREATMENT:** It is difficult to estimate the value of various remedies. General symptomatic and tonic treatment is indicated. Thyroid extract has seemed to benefit some cases. Local massage with oil or a mildly stimulating ointment is usually employed.