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Pemphiguserkrankungen: aktuelle und zukünftige Therapiekonzepte

Pemphigus: current and future treatment strategies

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A Publisher Erratum to this article was published on 08 January 2024

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Zusammenfassung

Pemphiguserkrankungen sind eine Gruppe organspezifischer Autoimmunerkrankungen, die durch die Produktion von Autoantikörpern gegen intraepidermale Adhäsionsmoleküle bzw. Strukturproteine der Haut und der hautnahen Schleimhäute verursacht werden. Klinisch zeigen sich je nach Krankheitsentität Blasen und Erosionen an Haut und/oder Schleimhäuten. Entsprechend der aktuellen AWMF(Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften e. V.)-S2k-Leitlinie zur Diagnostik und Therapie der Pemphiguserkrankungen wird eine systemische immunsuppressive bzw. immunmodulierende Therapie empfohlen. Die Systemtherapie setzt sich aus initial hoch dosierten Kortikosteroiden und einem steroidsparenden adjuvanten Immunsuppressivum, meist Azathioprin oder Mycophenolat-Mofetil, zusammen. In der Leitlinie wird bereits bei mildem Pemphigus vulgaris sowie bei moderatem bzw. schwerem Pemphigus foliaceus die Behandlung mit dem monoklonalen B‑Zell-depletierenden Anti-CD20-Antikörper Rituximab empfohlen.

Abstract

Pemphigus diseases are a group of organ-specific autoimmune diseases which are characterised by the production of autoantibodies against intra-epidermal adhesion molecules and structural proteins of skin and mucosae. Depending on the entity, patients develop blisters and erosions on the skin and/or mucosae. According to the AWMF S2k guidelines for diagnosis and therapy of pemphigus diseases, a systemic therapy is recommended. Initially, high-dose, oral corticosteroids in combination with immunosuppressive drugs as corticosteroid-sparing agent, usually azathioprine or mycophenolate mofetil, can be used. Furthermore, rituximab, a monoclonal antibody directed against CD20 on B cells, was recently approved for pemphigus vulgaris and moderate or severe pemphigus foliaceus.

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Förderung

Diese Arbeit wurde durch ein Grant der Deutschen Forschungsgemeinschaft (DFG, FOR 2497 Pegasus an Rüdiger Eming) unterstützt.

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  1. Klinik für Dermatologie und Allergologie, Philipps-Universität Marburg, Baldingerstr., 35043, Marburg, Deutschland

    Jacqueline Kussini, Antonio Manuel Sequeira Santos,  Rüdiger Eming &  Dario Didona

  2. Klinik III Dermatologie, Venerologie & Allergologie, Bundeswehrzentralkrankenhaus Koblenz, Koblenz, Deutschland

    Tabea Kaisers &  Rüdiger Eming

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  1. Jacqueline Kussini
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Kussini, J., Kaisers, T., Sequeira Santos, A.M. et al. Pemphiguserkrankungen: aktuelle und zukünftige Therapiekonzepte. Dermatologie 74, 915–926 (2023). https://doi.org/10.1007/s00105-023-05246-y

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