An Historical Account of the Study of Progressive Systemic Sclerosis (Diffuse Scleroderma)

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Abstract
Progressive systemic sclerosis (diffuse scleroderma) is typical of a number of the rheumatic diseases in that description of its cutaneous manifestation (scleroderma) long preceded awareness of systemic involvement. Recognition of the visceral lesions in this disorder followed upon advances in clinical and pathological diagnosis in the latter part of the 19th and early decades of the present century and much of the credit for this accomplishment rests upon the observations of early pathologists and roentgenologists. The present paper is concerned with the rather long and involved history of scleroderma (including its associations with cutaneous calcinosis, morphea, and Raynaud''s phenomenon), the early accounts of changes in the joints, lungs, esophagus and gastrointestinal tract, heart, and kidneys, and the pathologic studies of the relationship of progressive systemic sclerosis to other diseases of connective tissue.

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