title: Guidelines on dermatomyositis--excerpt from the interdisciplinary S2k guidelines on myositis syndromes by the German Society of Neurology.
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.
authors.affiliation: Department of Dermatology, University Hospital Münster, and Department of Translational Dermatoinfectiology, Medical Faculty of the University of Münster and University Hospital, Münster, Germany.
authors.forename: Cord
authors.initials: C
authors.lastname: Sunderkötter
authors.affiliation: Division of Evidence-based Medicine (dEBM), Department of Dermatology, Venereology, and Allergology, Charité - University Medical Center Berlin, Berlin, Germany.
authors.forename: Alexander
authors.initials: A
authors.lastname: Nast
authors.affiliation: Department of Dermatology, Venereology, and Allergology, Charité, Berlin, Germany.
authors.forename: Margitta
authors.initials: M
authors.lastname: Worm
authors.affiliation: Department of Neurology, Medical University Hanover, Hanover, Germany.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Dengler
authors.affiliation: Department of Medicine, Division of Rheumatology and Clinical Immunology, Charité - University Medical Center Berlin, German Rheumatism Research Center, Berlin, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Dörner
authors.affiliation: German Association for Muscular Dystrophy (Executive Director).
authors.forename: Horst
authors.initials: H
authors.lastname: Ganter
authors.affiliation: Institute for Clinical Neuroimmunology, Ludwig Maximilians University, Munich, Germany.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Hohlfeld
authors.affiliation: Medical Park Bad Rodach and Department of Neurology, University of Erlangen, Erlangen, Germany.
authors.forename: Arthur
authors.initials: A
authors.lastname: Melms
authors.affiliation: Department of Neurology, University Hospital Münster, Münster, Germany.
authors.forename: Nico
authors.initials: N
authors.lastname: Melzer
authors.affiliation: Department of Neurology, University Hospital Bern, Bern, Switzerland.
authors.forename: Kai
authors.initials: K
authors.lastname: Rösler
authors.affiliation: Department of Neurology, University Hospital Göttingen, Göttingen, Germany.
authors.forename: Jens
authors.initials: J
authors.lastname: Schmidt
authors.affiliation: Neuromuscular Center, Department of Neurology, University Hospital Bern, Bern, Switzerland.
authors.forename: Michael
authors.initials: M
authors.lastname: Sinnreich
authors.affiliation: Friedrich-Baur Institute, Ludwig Maximilians University, Munich, Germany.
authors.forename: Maggi C
authors.initials: MC
authors.lastname: Walter
authors.affiliation: Department of Neurology, University Hospital Innsbruck, Innsbruck, Austria.
authors.forename: Julia
authors.initials: J
authors.lastname: Wanschitz
authors.affiliation: Department of Neurology, University Hospital, Münster, Germany.
authors.forename: Heinz
authors.initials: H
authors.lastname: Wiendl
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D003880:Dermatology / Q000592:standards*; D003882:Dermatomyositis / Q000175:diagnosis* / Q000628:therapy*; D003943:Diagnostic Techniques, Neurological; D005858:Germany; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009462:Neurology / Q000592:standards*; D017410:Practice Guidelines as Topic
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents