Prognostic significance of Raynaud's phenomenon and other clinical characteristics of systemic scleroderma. A study of 271 cases.

THE CLASSIFICATION of seleroderma has been a controversial subject primarily because adequate follow-up studies have been lacking. It is generally agreed that localized forms of seleroderma such as morphea and linear seleroderma are not progressive or systemic diseases and have a good prognosis. The same is true for seleroderma confined to the fingers and toes of patients with long-standing Raynaud's disease (selerodactylia). It is in the classification of generalized or systemic seleroderma that confusion has arisen. In 1943, O'Leary and Waisman' advocated perpetuation of the term "acroselerosis" first proposed by Hutchinson2 and favored by Sellei.3 These workers considered acroselerosis to be a relatively benign and only slowly progressing form of generalized seleroderma with Raynaud's phenomenon as a constant and usually prominent clinical feature. The prognosis in acroselerosis was considered favorable when contrasted with that in "generalized progressive seleroderma," in which the cutaneous changes usually appeared first on the trunk rather than acrally and in which Raynaud's phenomenon was absent or minimal. Generalized progressive seleroderma was described by O 'Leary and Waisman as a fulminating disease, usually culminating in death within 2 years. In studies of Ravnaud 's phenomenon and Raynaud's disease among women and girls4 a many patients with Raynaud 's phenomenon