GENERALIZED SCLEREDEMA: REPORT WITH AUTOPSY FINDINGS

Abstract

103 cases of scleredema have been reported since 1900. The systemic nature of this disease has been recently proposed. The patient presented was seen 8 mos. after the onset of the disease and followed until her death 6 yrs. later. Treatment was nonspecific and ineffective. The pathologic changes in scleredema consist chiefly of thickening and hyalinization of the collagen bundles, with the production or deposition of a mucin-like material between the fibers. Since these changes occur throughout the body, it would seem logical to include this disease among those known as diseases of the collagen system. This is particularly applicable when one considers the fact that the pathogenesis of disease of the collagen system is probably concerned mainly with the intercellular substance. One previous autopsy report in this disease was probably a case of scleroderma. In the differential diagnosis the disease offering the greatest difficulty would be dermatomyositis. The absence of inflammatory changes explains the frequent remission of the systemic and skin changes.