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.1 Patients 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
—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
Schimke RN, Kirkpatrick CH, Delp MH. Calcinosis, Raynaud's Phenomenon, Sclerodactyly, and Telangiectasia: The CRST Syndrome. Arch Intern Med. 1967;119(4):365–370. doi:10.1001/archinte.1967.00290220115007
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: