Diagnosis and treatment of scleroderma.

Scleroderma is a rare disease. Approximately 80% of patients are females, and one-half present before the age of 40. Some studies suggest a higher incidence and severity of disease in black females than in whites. Scleroderma affect approximately 20 new patients per million per year and has an estimated prevalence of approximately 250 patients per million in the United States, the synonyms from this disease including Progressive systemic sclerosis (PSS), or diffuse scleroderma. Scleroderma is a multisystem disorder characterized by skin thickening and vascular abnormalities. Causes of scleroderma remain mysterious. Immunologic abnormalities are suggested by the presence of characteristic autoantibodies such as ANA,anticentromere, and anti-Scl-70 antibodies. In addition to skin, the most commonly affected organs are lung and kidney. Three major diseases subsets are recognized based on the extent of skin disease. Limited disease is defined as skin fibrosis in distal extremities and some areas of face and neck. Limited diseases are also known as CREST syndrome. Diffuse disease includes patients with skin abnormalities extending to the proximal extremities (i.e., above the elbow or knee) and trunk. Localized disease manifests as patches (morphea) or bandlike (linear scleroderma) areas of skin thickening.