Escleromixedema: um caso tratado com prednisona oral

Abstract

Scleromyxedema is an idiopathic cutaneous mucinosis characterized by a papular eruption, skin induration and paraproteinemia. Histologically, fibrolast proliferation can be observed in the upper dermis associated with a mucine deposition. Treatment is difficult and at present there is no totally effective therapeutic modality to control the disease. The present report is on a 68-year-old patient with scleromyxedema without systemic manifestation, who responded to oral steroid therapy.

Case for diagnosis. Lichen myxedematosus

“…It is commonly associated with monoclonal paraproteinemia 1-3 and is characterized by papular lesions associated with erythema and thick, diffuse scleroderma-like changes. 2 The normochromic or erythematous papules are stiff and grouped, ranging from 1 mm to 4 mm. They are symmetrically arranged, primarily on the back of the hands and fingers, the extensor surface of the arms, the face, the upper torso, and the legs.…”

“…1,4 They may coalesce, resulting in widespread induration of the skin and eventually leading to leonine facies and microstomia. 1,2 …”

“…1 Extracutaneous manifestations include dermato-neural syndrome, myopathy, inflammatory polyarthritis, esophageal disorders, changes in the larynx and nerves, pulmonary disease, and heart and liver abnormalities. 1,2 Cardiac abnormalities occur in 10% of cases, characterized by mucin deposition in the middle layer and adventitia of the myocardial vessels, as well as mucinous degeneration of the atheromatous plaques of the arteries. 5 Association with systemic hypertension has also been reported.…”

“…Histological exam of the upper dermis shows a horizontal band of mucinous material between the round, stellate-shaped and irregularly distributed collagen fibers and fusiform fibroblasts, 1,2 as well as dermal fibrosis. 3 …”

“…2 Alkylating agent melphalan was considered a first-line treatment, but limited use due to its side effects. 1-3 …”