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Dermatomyositis – update

Dermatomyositis—update

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Zusammenfassung

Die Dermatomyositis (DM) ist eine seltene idiopathische inflammatorische Myopathie und betrifft Kinder und Erwachsene, vorwiegend Frauen. Charakteristischerweise finden sich eine Myositis mit Nekrose, Regeneration und perifaszikulärer Atrophie und typische Hautveränderungen (Heliotrop- und Gottron-Erytheme, Gottron-Papeln und Nagelfalzveränderungen mit Splitterblutungen). Letztere können auch 6 Monate bis 2 Jahre vor der Muskelsymptomatik auftreten (amyopathische DM). Innerhalb eines klinisch heterogenen Spektrums erlauben Myositis-spezifische Autoantikörper (MSA) zunehmend klinisch-serologische Korrelationen. Alter, Autoantikörperprofile, Überlappung mit anderen Kollagenosen oder Vorkommen von Malignomen (paraneoplastische DM) bestimmen weitgehend Verlauf und Prognose. Die paraneoplastische DM betrifft Frauen und Männer etwa gleich häufig. Bei entsprechender immungenetischer Prädisposition können Infektionen, Medikamente oder Tumoren die Aktivierung von B- und T-Zellen, plasmazytoiden dendritischen Zellen, Überproduktion von Typ-I-Interferonen und Aktivierung des Komplementsystems mit Endothelzellschädigung induzieren. Auch UV-Exposition kann einen Schub triggern. Seit der Einführung hoch dosierter Kortikosteroide per os (1,5–2,0 mg/kg Körpergewicht/Tag) initial mit langsamer Reduktion, alleine oder in Kombination mit steroidsparenden Immunsuppressiva wie Azathioprin, Methotrexat, Mycophenolat-Mofetil oder mit hoch dosierten intravenösen Immunglobulinen hat sich die Prognose von 50 % Überlebensrate auf 90 % deutlich verbessert. Derzeit werden neue Therapieansätze mit Biologika (CD20-, TNF-α- und Interferon-Antikörper) und Kinaseinhibitoren evaluiert.

Abstract

Dermatomyositis is a rare idiopathic inflammatory myopathy that affects adults and children, mostly female. Hallmarks of the disease are myositis with necrosis, regeneration and perifascicular atrophy accompanied by a typical skin rash with heliotrope erythema, Gottron’s sign, Gottron’s papules and nail fold changes with splinter hemorrhage. Typical skin symptoms may appear 6 months up to 2 years before muscle involvement (amyopathic dermatomyositis). New myositis-specific antibodies may allow clinicoserologic correlations within a heterogeneous clinical spectrum. Autoantibody profiles, subtype of myositis, overlap with other collagen vascular disorders and/or malignancy (paraneoplastic dermatomyositis) as well as age of the patients all have a considerable impact on course and prognosis. Infections, drugs and tumors may trigger activation of T and B cells, plasmacytoid dendritic cells, overproduction of type I interferons and complement-mediated endothelial cell damage resulting in vasculopathy. UV radiation may also trigger dermatomyositis. Oral corticosteroids (1.5–2.0 mg/kg body weight/day) are the mainstay of treatment until improvement of muscle symptoms and/or normalization of muscle enzymes with subsequent slow tapering. Corticosteroids may be given as monotherapy or combined with steroid-sparing immunosuppressive agents‚ i.e. azathioprine, methotrexate, mycophenolate mofetil or high-dose intravenous immunoglobulins. Prognosis has improved considerably since use of high-dose corticosteroids, from 50 to 90 % response rate. New therapies with biologicals (anti-CD20-, anti-TNFalpha-, anti-interferon antibodies) and Janus kinase inhibitors are currently being evaluated.

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  1. Dermatologische Abteilung, Donauspital – Sozialmedizinisches Zentrum Ost Wien (Akademisches Lehrkrankenhaus der Medizinischen Universität Wien), Langobardenstrasse 122, 1220, Wien, Österreich

    B. Volc-Platzer

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Volc-Platzer, B. Dermatomyositis – update. Hautarzt 66, 604–610 (2015). https://doi.org/10.1007/s00105-015-3659-0

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