Intractable skin necrosis and interstitial pneumonia in amyopathic dermatomyositis, successfully treated with cyclosporin A.

We report a patient with amyopathic dermatomyositis (DM) who mainly showed interstitial pneumonia and intractable skin necrosis in bilateral elbows and soles with a poor response to immunomediated therapy, including corticosteroid and high-dose intravenous immunoglobulin. Soon after starting oral cyclosporin A (CyA) the skin lesions healed completely and the interstitial pneumonia promptly improved in parallel with a decrease in serum KL-6. Since fatal interstitial pneumonia is frequently associated with amyopathic DM as in this case, administration of CyA should be actively considered as a therapeutic option when clinical symtoms are progressive and resistant to conventional treatments.