Visceral involvement and Raynaud's phenomenon are rare. The clinical presentation evolves through three stages with various stages present simultaneously in different areas of the body. The last phase involves rippling of the skin with areas of hypopigmentation, induration and skin tightness. With elevation of involved extremities there may be furrows along the course of the superficial veins referred to as the groove sign. The hands, feet and face are spared.
Subclinical myositis is present in a minority of patients.
In contrast to scleroderma, Raynaud's phenomenon, abnormal nail fold capillaries and sclerodactly are not present. Visceral involvement is rare with few reports of involvement of the lungs, esophagus and myocardium. In addition, pancytopenia, anemia or thrombocytopenia may be encountered. Immunoglobulin levels show hypergammaglobulinemia, usually polyclonal IgG. Muscle enzymes are sometimes elevated, especially aldolase. ANA is occasionally positive.