Dermatomyositis is a disease that has a characteristic skin eruption that may occur with or without a proximal myopathy. The disease with cutaneous features only is classified as amyopathic dermatomyositis. The origin is unknown, but autoimmune factors are believed to play an important role. Autoantibodies are found in most patients and some have myositis-specific antibodies. Systemic changes may occur and there appears to be a relationship to internal malignancy, particularly in older patients. Juvenile disease has an associated vasculopathy. Treatment includes systemic corticosteroids and other immunosuppressive agents. The cutaneous changes may be difficult to treat.