Progressive Systemic Sclerosis

The visceral manifestations of scleroderma have been recognized for almost 60 years, but the relative infrequency of the disease and the difficulty of obtaining adequate pathological material have, with certain outstanding exceptions, precluded the pursuit of comprehensive studies. Beerman,1in an excellent review of the literature, stressed the protean nature of the disease which Becker and Obermayer2have ably defined as follows: Generalized scleroderma is not strictly a dermatosis, but rather a disease of the connective tissue of various organs and systems, the skin, subcutaneous tissue, muscles, bones and joints, tendons and fascias, serous surface, internal organs, endocrine glands and nervous system. The patient first notes the surface alterations, consequently the dermatologist is usually first consulted. Goetz3has suggested the term progressive systemic to describe the clinical picture until an etiology has been established. According to the author: Progressive systemic sclerosis