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Update: Dermatomyositis

Update: Dermatomyositis

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Zusammenfassung

Die Dermatomyositis gehört zu den seltenen inflammatorischen Myopathien mit einem heterogenen klinischen Spektrum. Typische Veränderungen sind periorbitale Heliotroperytheme, akrale Gottron-Papeln und proximale Muskelschwäche. Muskelspezifische Antikörper korrelieren mit bestimmten Myopathieverlaufsformen. Überlappungen mit anderen Kollagenosen kommen vor, und bei etwa einem Drittel finden sich Malignome (paraneoplastische Dermatomyositis). Therapie der ersten Wahl sind hoch dosierte Kortikosteroide per os bis zur erkennbaren Besserung der Muskelbeteiligung, des Weiteren steroidsparende Immunsuppressiva und hoch dosierte intravenöse Immunglobuline. Die Prognose hat sich durch die Therapie mit hoch dosierten Kortikosteroiden mit Ansprechraten bis zu 90% sprunghaft verbessert. Jährliche Kontrollen sind wegen des Malignomrisikos erforderlich.

Abstract

Dermatomyositis is an idiopathic inflammatory myopathy and an “orphan disease” (incidence 1:100,000). It comprises a heterogenous clinical spectrum with periorbital heliotrope erythema, acral Gottron papules, and proximal muscle weakness. Muscle-specific antibody profiles correlate with clinical variants. Overlap with other collagen vascular disorders occurs and about one-third of patients have an underlying malignancy (paraneoplastic dermatomyositis). High-dose oral corticosteroids are the mainstay of treatment, given until improvement of muscle symptoms and/or normalization of muscle enzymes Additional options include steroid-sparing immunosuppressants, or high dose intravenous immunoglobulins. Prognosis has improved considerably since use of high-dose corticosteroids, with about 90% of patients responding. Follow-up and search for a possible malignancy should be performed yearly.

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin: Abbott, Actelion, AESCA, Biotest, Janssen-Cilag, Intendis, Pelpharma, Roche, Wyeth, ZLB-Behring, Cephalon, Merck-Sorrano.

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Correspondence to B. Volc-Platzer.

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Volc-Platzer, B. Update: Dermatomyositis. Hautarzt 61, 69–80 (2010). https://doi.org/10.1007/s00105-009-1897-8

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  • DOI: https://doi.org/10.1007/s00105-009-1897-8

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