Systemic scleroderma, A clinical study of 727 cases.

Abstract

Scleroderma was described centuries ago, and yet today the physician has little to offer other than the diagnosis. The etiology remains obscure; the course is unpredictable, and therapy is of little benefit. The term is limited, for literally it means merely hard skin. Under this label are included disease processes varying from the sclerosis of a bit of skin to overwhelming systemic reactions terminating in rapid death. While innumerable case reports and reviews of scleroderma have appeared in the literature, we believed that a longterm clinical study, unique in the extremely large number of patients observed, would add to our knowledge of a poorly understood entity. Classification The classification of scleroderma used in the Section of Dermatology at the Mayo Clinic is as follows: Localized Morphea Generalized morphea Linear scleroderma En coup de sabre Hemiatrophy Systemic Acrosclerosis Diffuse scleroderma In this paper we will be concerned only with the