Abstract

Scleroderma had been virtually unrecognized in this country before this study. Our interest in this condition was raised by the discovery that certain patients being investigated for ischaemic disease of the hand had scleroderma. Although uncommon, it is not excessively rare and we have been able to study an increasingly large number of patients, eventually resulting in 177 patients over a period of 35 years. The clinical features in these patients have been delineated. At first, the patients were subdivided into types: type 1, skin changes obvious only in the hands; type 2, skin changes extending beyond the hands but excluding the trunk; type 3, skin changes diffuse and involving the trunk. All types have similar visceral changes, but these are more severe and there is a worse prognosis in type 3 patients. Types 1 and 2 can conveniently be combined as acrosclerosis. Types 1 and 2 have a similar and good prognosis with survival at 30 years of 40%. Type 3 patients have a much worse prognosis, with no type 3 patients living more than 20 years. All types have a high incidence of autoantibodies, but these are generally not related to the severity of the disease and do not occur in relatives or spouses, this being the evidence of the absence of hereditary and environmental factors in their presence. Although patients may receive much relief from symptomatic measures, no treatment had lessened the skin stiffness and there is no specific treatment for the visceral lesions. The cause of the condition remains unknown.