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Der Hautarzt

, Volume 69, Issue 1, pp 67–83 | Cite as

Schleimhautpemphigoid

  • M. M. HoltscheEmail author
  • D. Zillikens
  • E. Schmidt
CME

Zusammenfassung

Das Schleimhautpemphigoid (SHP) gehört zur Gruppe der Pemphigoiderkrankungen. Die Erkrankung ist definiert durch das Auftreten von Autoantikörpern gegen die dermoepidermale Junktionszone und den vorwiegenden Befall der Schleimhäute. Die Diagnose des SHP wird bei entsprechendem klinischem Phänotyp durch lineare Ablagerungen von IgG und/oder IgA und/oder Komplement C3 an der dermoepidermalen Junktionszone in der direkten Immunfluoreszenz einer periläsionalen Biopsie gestellt. Zirkulierende Autoantikörper lassen sich meist mittels indirekter Immunfluoreszenz auf humaner Spalthaut und ELISA („enzyme-linked immunosorbent assay“) oder Immunoblotuntersuchungen unter Verwendung zellulärer oder rekombinanter Antigene nachweisen. Zur systemischen Therapie des SHP werden Kortikosteroide, Dapson, Mykophenole und Azathioprin eingesetzt. Bei schweren Fällen und rascher Progression kommen zusätzlich Cyclophosphamid, Rituximab, hoch dosierte intravenöse Immunglobuline und Immunadsorption zum Einsatz. Entscheidend für die erfolgreiche Behandlung des SHP ist die Zusammenarbeit mit Kollegen anderer Fachrichtungen, wie Zahn-, Augen- und HNO-Ärzten sowie Gastroenterologen und Gynäkologen.

Schlüsselwörter

Schleimhauterosionen Autoantikörper Immunfluoreszenz Immunsuppression Basalmembranzone 

Mucous membrane pemphigoid

Abstract

Mucous membrane pemphigoid (MMP) is a pemphigoid disease defined by the presence of autoantibodies against the dermal–epidermal junction and predominant involvement of mucous membranes. Diagnosis is made by the clinical presentation and linear deposits of IgG and/or IgA and/or C3 at the dermal–epidermal junction by direct immunofluorescence microscopy of a perilesional biopsy. Circulating autoantibodies can be detected in most patients by indirect immunofluorescence microscopy on salt-split human skin as well as ELISA and immunoblotting with recombinant and cell-derived target antigens. For systemic treatment of MMP, corticosteroids, dapsone, mycophenolates, and azathioprine are applied. In severe cases and in cases with rapid disease progression cyclophosphamide, rituximab, high-dose intravenous immunoglobulins, and immunoadsorption are used. For the successful management of MMP patients, close cooperation with dentists, ophthalmologists, ENT specialists, gynecologists, and gastroenterologists is essential.

Keywords

Mucous membrane erosions Autoantibodies Immunofluorescence Immunosuppression Basal membrane zone 

Notes

Danksagung

Wir danken der Deutschen Forschungsgemeinschaft für die Unterstützung unserer Forschung zum Schleimhautpemphigoid im Exzellenz-Cluster 306/1 & 2 Inflammation at Interfaces und in der Klinischen Forschergruppe 306 Pemphigoid Diseases.

Einhaltung ethischer Richtlinien

Interessenkonflikt

M.M. Holtsche gibt an, dass kein Interessenkonflikt besteht. D. Zillikens und E. Schmidt haben eine wissenschaftliche Kooperation mit der Euroimmun AG, Lübeck.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren. Alle Patienten, die über Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts zu identifizieren sind, haben hierzu ihre schriftliche Einwilligung gegeben. Im Falle von nicht mündigen Patienten liegt die Einwilligung eines Erziehungsberechtigten oder des gesetzlich bestellten Betreuers vor.

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© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2017

Authors and Affiliations

  1. 1.Klinik für Dermatologie, Allergologie und VenerologieUniversität zu LübeckLübeckDeutschland

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