title: Response of mucocutaneous lesions to rituximab in a case of melanoma differentiation antigen 5-related dermatomyositis.
abstract: We report the first case in Western Europe of a person presenting with dermatomyositis associated with melanoma differentiation antigen 5 antibodies. She sequentially developed severe mucocutaneous erythematous and itchy lesions of the face, scalp, neck, knees and recurrent aphthae. In addition she presented painful, periungual, edematous digital lesions and small ulcers with digital necrosis. Her rapidly evolving, near-fatal interstitial lung disease responded to high-dose intravenous cyclophosphamide. However, her recurrent mucocutaneous manifestations improved only after rituximab administration.
authors: Immunology and Allergy, University Hospital and School of Medicine, Geneva, Switzerland.
authors: Aurélie
authors: A
authors: Clottu
authors: Emmanuel
authors: E
authors: Laffitte
authors: Christa
authors: C
authors: Prins
authors: Carlo
authors: C
authors: Chizzolini
mesh_terms: D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001323:Autoantibodies / Q000097:blood*; D053487:DEAD-box RNA Helicases / Q000276:immunology*; D003882:Dermatomyositis / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D000072640:Interferon-Induced Helicase, IFIH1; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D001323:Autoantibodies; D000069283:Rituximab; C452815:IFIH1 protein, human; D053487:DEAD-box RNA Helicases; D000072640:Interferon-Induced Helicase, IFIH1