Dermatomyositis

Marinos C Dalakas MD (Dr. Dalakas of the University of Athens Medical School and Thomas Jefferson University received honorariums from Baxter, Hoffmann LaRoche, Novartis, and Therapath for consulting, serving on advisory committees, and speaking engagements.)
Salvatore DiMauro MD, editor. (Dr. DiMauro, Director Emeritus of H Houston Merritt Clinical Center for the Study of Muscular Dystrophy and Related Diseases at Columbia University, has no relevant financial relationships to disclose.)
Originally released September 14, 1995; last updated July 24, 2016; expires July 24, 2019

Historical note and terminology

Dermatomyositis is 1 of the 5 main clinicopathologically distinct inflammatory myopathies, the other 4 being (1) polymyositis, (2) necrotizing autoimmune myositis, (3) overlap myositis, and (4) inclusion-body myositis (Dalakas 2015). Although it may have been recognized earlier by Wagner (Wagner 1863), the first definitive description of dermatomyositis was reported by Unverricht in 1891 (Unverricht 1891). Before the extensive use of immunosuppressive drugs, dermatomyositis was the cause of considerable disability and mortality, especially in children. During the last 30 years, extensive use of these drugs and a better understanding of the immunopathology of the disease have significantly improved the prognosis. Dermatomyositis, a disease affecting skin and muscle, is cared for not only by neurologists but also by rheumatologists and dermatologists. The role of the neurologist is essential to exclude other neuromuscular diseases associated with skin abnormalities, to establish the cause of muscle weakness, to confirm the immunopathology of the disease, and to supervise the immunotherapeutic interventions. In children with dermatomyositis with muscle weakness as the major symptom, the pediatric neurologist is often consulted early in the disease and usually becomes the primary physician caring for these children. The verb “present” has become ambiguous, sometimes referring to the disease, sometimes to the patient. When it refers to the patient, it indicates the first symptoms or the reason for the first medical consultation.

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