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Seltene Varianten der Pemphigoiderkrankungen

Rare variants of pemphigoid diseases

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

This article has been updated

Zusammenfassung

Pemphigoiderkrankungen umfassen eine heterogene Gruppe subepidermal blasenbildender Autoimmundermatosen, die durch Autoantikörper gegen Strukturproteine der dermoepidermalen Junktionszone gekennzeichnet sind. In den letzten Jahrzehnten ist eine deutlich steigende Inzidenz dieser Erkrankungen zu verzeichnen, was neben demografischer Alterung auf die Verfügbarkeit präziser Diagnoseverfahren und verbesserte Kenntnis des klinischen und immunpathologischen Spektrums zurückgeführt werden kann. Während das bullöse Pemphigoid, das Schleimhautpemphigoid und die lineare IgA-Dermatose einen Großteil der Pemphigoiderkrankungen ausmachen, rücken auch seltenere, vermutlich unterdiagnostizierte Varianten zunehmend in den klinischen Fokus. Hierzu zählen die Epidermolysis bullosa acquisita, das Anti-p200-Pemphigoid, das Pemphigoid gestationis, der Lichen planus pemphigoides und kürzlich definierte Entitäten wie das Immunglobulin M(IgM)-Pemphigoid und das Orf-induzierte Pemphigoid. Die genaue Charakterisierung und Differenzierung dieser Subtypen besitzen nicht nur eine diagnostische Relevanz, sondern können auch mit therapeutischen und prognostischen Implikationen für betroffene Individuen einhergehen. Aufgrund der Rarität dieser Erkrankungen existieren derzeit keine kontrollierten prospektiven klinischen Studien, sodass deren Diagnostik und Therapie eine Herausforderung darstellen.

Abstract

Pemphigoid diseases comprise a heterogeneous group of subepidermal autoimmune blistering dermatoses characterized by autoantibodies against structural proteins of the dermal–epidermal junction. Recent decades have witnessed a significant surge in the incidence of these diseases, which, in addition to general aging of the population, can be attributed to the availability of precise diagnostic methods and improved knowledge of the clinical and immunopathological spectrum. While bullous pemphigoid, mucous membrane pemphigoid, and linear IgA disease account for most pemphigoid disorders, less frequent, presumably underdiagnosed variants are increasingly becoming relevant for clinicians. These include epidermolysis bullosa acquisita, anti-p200 pemphigoid, pemphigoid gestationis, lichen planus pemphigoides, and recently defined entities such as IgM pemphigoid and Orf-induced pemphigoid. Accurate characterization and differentiation of these subtypes are not only of diagnostic relevance but may also be associated with therapeutic and prognostic implications for affected individuals. Due to the rarity of these diseases, no controlled prospective clinical trials currently exist, making their diagnosis and therapy challenging.

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Danksagung

Wir bedanken uns bei der Deutschen Forschungsgemeinschaft für die Unterstützung unserer Forschung zu Pemphigoiderkrankungen im Sonderforschungsbereich (SFB) 1526 Pathomechanisms of Antibody-mediated Autoimmunity (PANTAU): Insights from Pemphigoid Diseases.

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  1. Klinik für Dermatologie, Allergologie und Venerologie, Universität zu Lübeck, Ratzeburger Allee 160, 23562, Lübeck, Deutschland

    Kaan Yilmaz, Charlotte Kiehne, Maike M. Holtsche &  Enno Schmidt

  2. Klinik für Dermatologie, Venerologie und Allergologie, Medizinische Fakultät Mannheim der Universität Heidelberg, Mannheim, Deutschland

    Kaan Yilmaz

  3. Klinik für Dermatologie und Allergologie, Universität Oldenburg, Oldenburg, Deutschland

    Maike M. Holtsche

  4. Lübecker Institut für Experimentelle Dermatologie, Universität zu Lübeck, Lübeck, Deutschland

    Enno Schmidt

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Correspondence to Enno Schmidt.

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E. Schmidt hat wissenschaftliche Kooperationen mit Euroimmun, UCB, Incyte, Biotest, ArgenX, Dompe, Admirx, Biondis, Fresenius Medical Care, Pharmaxis, Alpine Immune, Bayer, CSL, AstraZeneca und PinCell und Patente mit Euroimmun und Dompe. E. Schmidt bekam Honorare und/oder Reisekostenerstattung von Biotest, ArgenX, Fresenius Medical Care, AstraZeneca, Leo, Chugai, Sanofi und Almirall. K. Yilmaz, C. Kiehne und M.M. Holtsche geben an, dass kein Interessenkonflikt besteht.

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Yilmaz, K., Kiehne, C., Holtsche, M.M. et al. Seltene Varianten der Pemphigoiderkrankungen. Dermatologie 74, 937–947 (2023). https://doi.org/10.1007/s00105-023-05242-2

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