The onset of scleroderma [progressive systemic sclerosis (PSS)] is insidious. Patients often complain first of Raynaud’s phenomenon. In this condition the skin is taut and bound down and is shiny and may become dyspigmented. The disease may be limited or diffuse. The limited form of PSS is more common and is characterized by a slowly progressive hardening of the acral skin, a condition known as sclerodactyly (Fig. 67-1). In diffuse PSS, sclerodactyly is accompanied by widespread involvement of both the skin and the internal organs. The systemic symptoms include fatigue, dysphagia, dyspnea, abnormal bowel function, arthralgias, and myalgias. Hypertension may be caused by PSS involving the kidneys. A variant, known as the CREST syndrome, is characterized by calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.