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12.03.2019 | Glucocorticoide | Leitthema

Anti-p200-Pemphigoid

verfasst von: Dr. M. M. Holtsche, S. Goletz, D. Zillikens

Erschienen in: Die Dermatologie | Ausgabe 4/2019

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Zusammenfassung

Das Anti-p200-Pemphigoid ist eine seltene blasenbildende Autoimmundermatose. Es handelt sich um eine Erkrankung der Untergruppe der Pemphigoiderkrankungen und wurde erstmals 1996 beschrieben. Der Goldstandard in der Diagnostik ist die direkte Immunfluoreszenz (IF) einer periläsionalen Probebiopsie in Kombination mit dem Nachweis zirkulierender Autoantikörper mit Bindung an die dermale Seite (Blasenboden) humaner Spalthaut in der indirekten IF sowie Reaktivität mit einem 200 kDa schweren Protein (p200) im Immunoblot mit Extrakt humaner Dermis. Im Jahr 2009 wurde Laminin γ1 als zusätzliches Zielantigen in 90 % der Anti-p200-Pemphigoid-Patienten beschrieben. Da sowohl Ex-vivo- als auch In-vivo-Studien keine pathogene Relevanz von Laminin γ1 nachweisen konnten und die Präadsorption gegen Laminin γ1 die Reaktivität von Patientenseren mit p200 nicht reduzierte, bleibt die molekulare Identität des p200-Proteins weiterhin unklar. Das klinische Bild der Erkrankung ist vielseitig, in den meisten Fällen ähnelt es dem des bullösen Pemphigoids. Die Patienten sind jedoch im Durchschnitt etwas jünger und Läsionen finden sich häufiger an Palmae und Plantae als beim bullösen Pemphigoid. Therapeutisch wurden lokale und systemische Glukokortikosteroide sowie Dapson und Immunsuppressiva beschrieben.

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Titel: Anti-p200-Pemphigoid
verfasst von: Dr. M. M. Holtsche
S. Goletz
D. Zillikens
Publikationsdatum: 12.03.2019
Verlag: Springer Medizin
Schlagwörter: Glucocorticoide
Pemphigoiderkrankungen
Bullöses Pemphigoid
Schleimhautpemphigoid
Anti-p200-Pemphigoid
Epidermolysis bullosa acquisita
Erschienen in: Die Dermatologie / Ausgabe 4/2019
Print ISSN: 2731-7005
Elektronische ISSN: 2731-7013
DOI: https://doi.org/10.1007/s00105-019-4376-x

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