Dermatomyositis presenting with severe subcutaneous edema: Five additional cases and review of the literature

• Published in: Seminars in arthritis and rheumatism Vol. 44; no. 2; pp. 228 - 233
• Main Authors: Milisenda, José C., MD, Doti, Pamela I., MD, Prieto-González, Sergio, MD, Grau, Josep M., MD, PhD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.10.2014
• Subjects: Aged; Aged, 80 and over; Dermatomyositis; Dermatomyositis - complications; Dermatomyositis - diagnosis; Dermatomyositis - drug therapy; Edema - diagnosis; Edema - drug therapy; Edema - etiology; Fatal Outcome; Female; Generalized edema; Humans; Immunoglobulins, Intravenous - therapeutic use; Immunosuppressive Agents - therapeutic use; Inflammatory myopathy; Limb edema; Male; Middle Aged; Prednisone - therapeutic use; Retrospective Studies; Rheumatology; Severity of Illness Index; Skin Diseases - diagnosis; Skin Diseases - drug therapy; Skin Diseases - etiology; Treatment Outcome; Generalized edema; Inflammatory myopathy; Dermatomyositis; Limb edema
• ISSN: 0049-0172; 1532-866X
• DOI: 10.1016/j.semarthrit.2014.04.004

Abstract Objectives Dermatomyositis (DM) constitutes a subset of idiopathic inflammatory myopathies clinically characterized by proximal muscle weakness and skin involvement. Some of the dermatologic manifestations are highly prevalent and characteristic, but others such as generalized or limb edema are truly rare. The aim of the present study was to describe five cases of edematous DM diagnosed at our institution and to perform a review of the literature, as well as identify clinical, laboratory, or pathological data associated with this manifestation. Methods We performed a retrospective clinical, laboratory, and pathological evaluation of five cases of this edematous presentation out of 86 DM cases diagnosed at our hospital from 2004 to 2013. Moreover, we undertook a medical literature search using inflammatory myopathy, dermatomyositis, and edema as key words, limited to articles published in MEDLINE, EMBASE, and LILACS database in English and Spanish from 1987 to 2013. Results A total of 19 patients were identified, five diagnosed at our hospital and 14 cases from the literature. Overall, the median time from disease onset to diagnosis was 2 months, and most of the patients (16/84 patients, 21%) required more aggressive therapy, including immunosuppressive agents and intravenous immunoglobulin (12/63 patients, 15%). Microinfarction was present 2.3 times more frequently in DM patients with edema compared with those without edema. Conclusions The presence of edema in DM is uncommon but seems to be a sign of severe disease, requiring early and aggressive treatment. Microischemia-producing microinfarction may play an important pathophysiological role and determine the degree of disease severity.