Unilateral Eyelid Swelling as a Sign of Antimelanoma Differentiation-Associated Gene 5 (Anti-MDA5)-Antibody-Positive Dermatomyositis

摘要

The authors describe a 40-year-old woman who presented with unilateral eyelid swelling, and later on developed heliotrope rash, extensive oral and cutaneous ulcers, and muscle weakness. She was diagnosed with dermatomyositis associated with antimelanoma differentiation-associated gene 5 antibody. Even after combination of immunosupressants including a corticosteroid, tacrolimus, and rituximab, her eyelid swelling and oral ulcers progressively worsened. Administration of loading and then monthly intravenous immunoglobulin resulted in clinical remission, suggesting that intravenous immunoglobulin could be a useful remedy in cases refractory to conventional immunosuppressive agents, especially those with antimelanoma differentiation-associated gene 5 antibody-dermatomyositis.