Progressive Systemic Sclerosis Sine Scleroderma

Abstract

Four patients, a man aged 51 and 3 women aged 59, 69, and 71, died of progressive systemic sclerosis and yet had minimal or no evidence of cutaneous disease (scleroderma). In addition to illustrating the great variability in the clinical course of progressive systemic sclerosis, these cases serve to point up the difficulties involved in the recognition of this condition prior to the development of typical skin changes. When patients are encountered with such problems as unexplained esophageal or intestinal hypomotility and malabsorption, or both, myocardial disease, and pulmonary fibrosis, the diagnosis of progressive systemic sclerosis should be suspected from certain characteristic clinical and roentgenographic findings, and may be confirmed, at least in those with intestinal disease, by the histologic examination of tissue obtained at operation.