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      Guidelines on dermatomyositis--excerpt from the interdisciplinary S2k guidelines on myositis syndromes by the German Society of Neurology.

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          Abstract

          The present guidelines on dermatomyositis (DM) represent an excerpt from the interdisciplinary S2k guidelines on myositis syndromes of the German Society of Neurology (available at www.awmf.org). The cardinal symptom of myositis in DM is symmetrical proximal muscle weakness. Elevated creatine kinase, CRP or ESR as well as electromyography and muscle biopsy also provide important diagnostic clues. Pharyngeal, respiratory, cardiac, and neck muscles may also be affected. Given that approximately 30% of patients also develop interstitial lung disease, pulmonary function tests should be part of the diagnostic workup. Although the cutaneous manifestations in DM are variable, taken together, they represent a characteristic and crucial diagnostic criterion for DM. Approximately 5-20% of individuals exhibit typical skin lesions without any clinically manifest muscle involvement (amyopathic DM). About 30% of adult DM cases are associated with a malignancy. This fact, however, should not delay the treatment of severe myositis. Corticosteroids are the therapy of choice in myositis (1-2 mg/kg). Additional immunosuppressive therapy is frequently required (azathioprine, for children methotrexate). In case of insufficient therapeutic response, the use of intravenous immunoglobulins is justified. The benefit of rituximab has not been conclusively ascertained yet. Acute therapeutic management is usually followed by low-dose maintenance therapy for one to three years. Skin lesions do not always respond sufficiently to myositis therapy. Effective treatment for such cases consists of topical corticosteroids and sometimes also calcineurin inhibitors. Systemic therapies shown to be effective include antimalarial agents (also in combination), methotrexate, and corticosteroids. Intravenous immunoglobulins or rituximab may also be helpful. UV protection is an important prophylactic measure.

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          Author and article information

          Journal
          J Dtsch Dermatol Ges
          Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
          Wiley-Blackwell
          1610-0387
          1610-0379
          Mar 2016
          : 14
          : 3
          Affiliations
          [1 ] Department of Dermatology, University Hospital Münster, and Department of Translational Dermatoinfectiology, Medical Faculty of the University of Münster and University Hospital, Münster, Germany.
          [2 ] Division of Evidence-based Medicine (dEBM), Department of Dermatology, Venereology, and Allergology, Charité - University Medical Center Berlin, Berlin, Germany.
          [3 ] Department of Dermatology, Venereology, and Allergology, Charité, Berlin, Germany.
          [4 ] Department of Neurology, Medical University Hanover, Hanover, Germany.
          [5 ] Department of Medicine, Division of Rheumatology and Clinical Immunology, Charité - University Medical Center Berlin, German Rheumatism Research Center, Berlin, Germany.
          [6 ] German Association for Muscular Dystrophy (Executive Director).
          [7 ] Institute for Clinical Neuroimmunology, Ludwig Maximilians University, Munich, Germany.
          [8 ] Medical Park Bad Rodach and Department of Neurology, University of Erlangen, Erlangen, Germany.
          [9 ] Department of Neurology, University Hospital Münster, Münster, Germany.
          [10 ] Department of Neurology, University Hospital Bern, Bern, Switzerland.
          [11 ] Department of Neurology, University Hospital Göttingen, Göttingen, Germany.
          [12 ] Neuromuscular Center, Department of Neurology, University Hospital Bern, Bern, Switzerland.
          [13 ] Friedrich-Baur Institute, Ludwig Maximilians University, Munich, Germany.
          [14 ] Department of Neurology, University Hospital Innsbruck, Innsbruck, Austria.
          [15 ] Department of Neurology, University Hospital, Münster, Germany.
          Article
          10.1111/ddg.12909
          26972210

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