Dr. Dalakas of the National and Kapodistrian University of Athens Medical School in Greece and Thomas Jefferson University, Philadelphia, Pennsylvania, received honorariums from Baxalta (Shire), Novartis, Pfizer, CSL, and Octapharma for consulting, serving on advisory committees, and speaking engagements.)
Dr. Ciafaloni of the University of Rochester received personal compensation for serving on advisory boards and/or as a consultant for Avexis, Biogen, Pfizer, PTC Therapeutics, Sarepta, Ra pharma, Wave, and Strongbridge Biopharma; and for serving on a speaker’s bureau for Biogen. Dr Ciafaloni also received research and/or grant support from Orphazyme, PTC Therapeutics, Santhera, and Sarepta.)
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 the antisynthetase syndromes, 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 20 to 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 content you are trying to view is available only to logged in, current MedLink Neurology subscribers.
If you are a subscriber, please log in.
If you are a former subscriber or have registered before, please log in first and then click select a Service Plan or contact Subscriber Services. Site license users, click the Site License Acces link on the Homepage at an authorized computer.