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14.12.2017 | Schleimhautpemphigoid | CME

Schleimhautpemphigoid

verfasst von: M. M. Holtsche, D. Zillikens, E. Schmidt

Erschienen in: Die Dermatologie | Ausgabe 1/2018

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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.

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Titel: Schleimhautpemphigoid
verfasst von: M. M. Holtsche
D. Zillikens
E. Schmidt
Publikationsdatum: 14.12.2017
Verlag: Springer Medizin
Schlagwörter: Schleimhautpemphigoid
Bullöses Pemphigoid
Pemphigoiderkrankungen
Erschienen in: Die Dermatologie / Ausgabe 1/2018
Print ISSN: 2731-7005
Elektronische ISSN: 2731-7013
DOI: https://doi.org/10.1007/s00105-017-4089-y

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