A 48-year-old man presented with lower extremity weakness and rash. Physical examination revealed symmetrical proximal muscle weakness, extremity erythematous plaques, Gottron papules, and periorbital edema with a heliotrope hue. Serology revealed elevated titers of aminotransferases, aldolase, creatine kinase, and erythrocyte sedimentation rate. Skin biopsy suggested acute collagen vascular disease, and electromyography revealed proximal irritable myopathy. Muscle biopsy of the quadriceps was performed; however, the limited muscle tissue obtained failed to show signs of inflammation and the negative result was attributed to sampling error. The patient fulfilled the Bohan and Peter criteria for dermatomyositis (DM) based on clinical presentation and characteristic electromyography findings. His initial total lymphocyte count was 784/mm 3 , and his CD4 cell count was 318/mm 3 .
The patient responded to 60 mg/day of prednisone over a period of 7 days, and was discharged with nearcomplete recovery of muscle strength and decreased serum levels of muscle enzymes and aminotransferases. However, 2 weeks later, he again presented with profound dyspnea, fever, cough, tachycardia, bilateral knee pain, and facial swelling. Arterial blood gas analysis revealed hypoxemia and respiratory alkalosis. Chest radiography was normal, but chest computed tomography (CT) scan showed diffuse pleural thickening with extensive peripheral reticular nodular infiltrates. The patient received antibiotics for possible hospital-acquired pneumonia. Despite treatment, he continued to complain of dyspnea associated with rapid respiratory deterioration, and he ultimately required intubation. His total lymphocyte count was 300/mm 3 , and his CD4 cell count was 259/mm 3 . Bronchoscopy and bronchoalveolar lavage revealed Pneumocystis jirovecii organisms by staining and cytopathology. Subsequently, he was treated with intravenous trimethoprim-sulfamethoxasole and prednisone for Pneumocystis carinii pneumonia (PCP). His clinical status remained tenuous for several days, and he died of progressive respiratory failure.