Calcinosis, Raynaud's Phenomenon, Sclerodactyly, and Telangiectasia

SCLERODERMA or progressive systemic sclerosis is recognized as a disorder of connective tissue characterized by inflammation, degeneration, and fibrosis. Although the disease may be chronic, the involvement of specific organ systems varies widely. Recently, a variant of scleroderma, called the CRST syndrome, has been described.¹Patients with this disorder have skin changes such as dermal and subcutaneous calcinosis, Raynaud's phenomenon, sclerodactyly, and telangiectasia. With the possible exception of the gastrointestinal system, involvement of other organs is uncommon, and affected individuals have a long clinical course. This report describes three additional patients with the CRST syndrome. Of further interest is the fact that one of the patients has a family history of scleroderma, the fourth such occurrence recorded in the literature.

Report of Cases

Case 1.

—A 56-year-old white woman was admitted for the first time in May 1964, for evaluation of persistent fever. In February 1964, she had