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Lupus erythematodes

Lupus erythematosus

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Zusammenfassung

Hintergrund

Der Lupus erythematodes ist eine Autoimmunerkrankung mit breitem Spektrum kutaner Manifestationsformen. Der pathogenetisch zugrunde liegende Toleranzverlust gegenüber körpereigenen Antigenen kann durch Störungen der Apoptose, der Elimination von Zellresten und gesteigerte Aktivierung des angeborenen sowie adaptiven Immunsystems bedingt sein. Die gesteigerte Aktivität des angeborenen Immunsystems kann durch Stimulation mit endogenen oder exogenen Nukleinsäuren, genetischen Varianten in Komponenten der Rezeptorkaskaden oder Störungen der Nukleinsäurerestriktion induziert werden. Das resultierende Entzündungsmilieu wird wesentlich durch Typ-I-Interferone bestimmt und ist durch eine Autoantikörperproduktion gekennzeichnet. Wesentliche Provokationsfaktoren des Lupus erythematodes sind UV-Strahlung und Infektionen.

Therapie

Therapeutisch werden Glukokortikoide sehr wirksam eingesetzt. Zugelassene Alternativen für die Langzeittherapie sind Antimalariamedikamente und der B-Zell-Inhibitor Belimumab für Patienten mit systemischem Lupus erythematodes.

Schlussfolgerung

Zukünftige Studien sollten stärker den Effekt neuer Therapien auf den kutanen Phänotyp analysieren, damit frühzeitig ein Einsatz bei kutaner Manifestation des Lupus möglich wird. Zudem sind neue Therapiestrategien erforderlich, die gezielt auf die pathogenetisch verantwortlichen Mechanismen individueller Subtypen des Lupus erythematodes gerichtet sind, um so den Behandlungserfolg innerhalb der heterogenen Patientenpopulation zu verbessern.

Abstract

Background

Lupus erythematosus is an autoimmune disease with a broad spectrum of cutaneous manifestations. The pathogenesis of lupus is based on a loss of tolerance against self antigens and can be mediated by defects in apoptosis, defects in eliminating cellular remnants and increased activation of the innate as well as the adaptive immune system. The increased activation of the innate immune system can be mediated by sensing of endogenous or exogenous nucleic acids, genetic variants in the components of the receptor cascade or disturbances in restriction of self nucleic acids. The inflammatory milieu is characterized by type I interferon expression and autoantibody production. The main trigger factors of the disease are sun exposure and viral infections.

Treatment

Lupus erythematosus is effectively treated by glucocorticosteroids. Approved alternatives for long-term treatment are antimalarial agents and the B-cell inhibitor belimumab for patients with systemic lupus erythematosus.

Conclusion

Future studies should more intensely analyse the effect of novel therapies on cutaneous manifestations to allow early detection of cutaneous lupus. Furthermore novel therapeutic strategies which specifically target the responsible pathogenetic mechanisms of the individual subtypes of lupus erythematosus are needed to improve the therapeutic success for this heterogeneous patient population.

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Danksagung

C. Günther erhielt eine Förderung der Deutschen Forschungsgemeinschaft (GU1212/1-1 und GU 1212/1-2).

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  1. Klinik und Poliklinik für Dermatologie, Universitätsklinikum Dresden, Fetscherstraße 74, 01307, Dresden, Deutschland

    C. Günther & S. Beissert

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Correspondence to C. Günther.

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C. Günther und S. Beissert geben an, dass kein Interessenkonflikt besteht.

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Günther, C., Beissert, S. Lupus erythematodes. Hautarzt 66, 611–616 (2015). https://doi.org/10.1007/s00105-015-3644-7

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