A 34-Year-Old Man With Amyopathic Dermatomyositis and Rapidly Progressive Dyspnea With Facial Swelling

Abstract

A 34-year-old man was hospitalized for odynophagia, facial swelling, and progressive dyspnea. Sixteen months earlier, a violaceous rash had developed over extensor surfaces of the metacarpophalangeal and interphalangeal finger joints, elbows, and knees with periorbital swelling and an erythematous rash. He denied muscle weakness but noted cough and exertional dyspnea. Serum creatine kinase and aldolase levels and electromyography findings were normal. Antinuclear antibody and anti-Jo-1 antibody test results were negative. Skin biopsy demonstrated vascular ectasia alternating with vascular dropout. The findings of a chest radiograph and chest CT scan were normal. Amyopathic dermatomyositis was diagnosed, and therapy consisting of prednisone with azathioprine was begun.