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Arthritis und Rheuma 2013; 33(01): 25-32
DOI: 10.1055/s-0037-1618163
Kinderrheumatologie: Übersichtsarbeit
Schattauer GmbH

Juvenile Dermatomyositis

Juvenile Dermatomyositis
F. Dressler
1   Kinderklinik der Medizinischen Hochschule Hannover
,
A. Thon
1   Kinderklinik der Medizinischen Hochschule Hannover
› Author Affiliations
Further Information

Publication History

Publication Date:
27 December 2017 (online)

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Zusammenfassung

Die juvenile Dermatomyositis (JDM) ist eine entzündliche Multisystemerkrankung unklarer Ätiologie mit charakteristischen Manifestationen an Haut und quergestreifter Muskulatur und einem Beginn unter 18 Jahren. Auf ein Prodromalstadium folgt eine progressive proximal betonte Muskelschwäche. An der Haut sind die klassischen Zeichen der heliotrope Ausschlag, ein Wangenerythem, das Gottron-Zeichen und Nagelbettgefäßveränderungen. Zur Diagnose werden modifizierte Kriterien nach Bohan und Peter eingesetzt, die neben der klinischen Haut- und Muskelbeteiligung erhöhte Muskelenzymwerte und typische Befunde in Elektromyografie, Muskelbiopsie und neuerdings auch Magnetresonanztomografie beinhalten. Hochdosierte orale Steroide sind die klassische Behandlung. Intravenöse Steroidpulse mit niedrigerer oraler Intervalltherapie und Immunsuppressiva wie Methotrexat können Steroidnebenwirkungen reduzieren. Die Prognose der JDM hat sich verbessert. Die meisten Patienten erholen sich, aber einzelne Patienten versterben und eine Minderheit behält Krankheitsfolgen mit persistierender Muskelatrophie oder ausgeprägter Kalzinose.

Summary

Juvenile dermatomyositis (JDM) is an inflammatory multi-system disease with unknown etiology and classic skin and striated muscle manifestations and onset before 18 years of age. After a prodromal period patients develop progressive proximal muscle weakness. Typical skin involvement includes heliotrope rash, facial erythema, Gottron’s sign and nailfold capillary abnormalities. To diagnose JDM modified Bohan and Peter criteria are used including clinical skin and muscle signs plus elevated muscle enzymes and typical findings on electromyography, muscle biopsy and more recently also on magnetic resonance imaging. High-dose oral steroids are the classic treatment. Intravenous steroid pulse therapy with intermittent lower dose oral steroids and other immunosuppressive drugs such as methotrexate reduce steroid side-effects. Prognosis in JDM has improved, and most patients eventually recover fully. However, a few patients die from the disease, and in a minority significant damage with muscle atrophy or severe calcinosis results.

Schlüsselwörter

Juvenile Dermatomyositis - Kalzinose

Keywords

Juvenile dermatomyositis - calcinosis
 

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