This clinical report concerns a 57 year old woman with dermatomyositis and cardiac involvement. After an apparent clinical and biological improve of the muscular and myocardic symptoms, a pulmonary involvement was reported. There was a persistent high rate of lacticodeshydrogenase serum activity. Then, cutaneous ulcerations and necrosis appeared on the extremities of limbs. Dermatomyositis associated with cutaneous necrosis and pulmonary fibrosis has a very severe prognosis.