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Dermatomyositis is one of the idiopathic inflammatory myopathies with characteristic cutaneous manifestations including the heliotrope rash, Gottron's papules, cuticular changes including perlungual telanglectasia, a photodistributed erythem a or poliloderma, and a scaly alopecia. Dermatomyositis has been linked to cancer, particularly ovarian cancer. Cancer-associated disease is more commonly found in older patients, and when present, is associated with a poor prognosis. A childhood form of the disease exists and is frequently complicated by the development of calcinosis. Dermatomyositis is a systemic disorder and whereas the skin and muscles are the most commonly affected organs, patients may have arthralgias, arthritis, oesophageal dis ease, or cardiopulmonary dysfunction. Recently described serological abnormalities, known as myositis-specific antibodies, add credence to the notion that this disorder is distinct form all other collagen-vascular diseases, and may lead to important disc overies about the pathogenesis of the inflammatory myopathies, which are not currently of practical use in the clinic or office. Management of the patient with myositis usually includes systemic corticosteroids with or without an immunosuppressive agent. Cutaneous disease is more difficult to manage, but antimalarials, methotrexate,and intravenous immunoglobulin are effective in small, often open-label studies. |
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