Dermatomyositis in childhood.

Childhood dermatomyositis may be a serious and even lethal disease of childhood. Two types are recognized: the rare Banker type, based on vasculopathy and infarction commonly proceeding to death, and the more common Brunsting type, which is a steroid-responsive inflammatory myopathy comparable to the usual adult form. The diagnosis is suggested by the finding of skin changes (present in most patients) and by the results of clinical, enzymatic and electromyographic evaluation of muscle function. A muscle biopsy specimen should be taken in all cases to observe muscle and vessel changes and to rule out unusual myopathy. Direct immunofluorescence of skin and muscle may be helpful. Steroid therapy has reduced inflammation, morbidity and mortality, but remissions may occur without treatment. Methotrexate may be used when there is no response to steroids. Nutrition and physical therapy must be applied properly to be effective. Corticosteroid therapy of the disease has improved the prognosis for musculoskeletal function and for life.