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Krankheiten mit intraepidermalen Blasen und Pusteln

  • D. Metze

Zusammenfassung

Der Pemphigus (P.) vulgaris ist die häufigste Erkrankung aus der Pemphisgusgruppe. Der Altersgipfel liegt in der 4. bis 6. Lebensdekade. Es besteht keine Geschlechtsprävalenz, aber eine ethnische mit Häufung bestimmter HLA-Merkmale. Selten wurde die Assoziation mit anderen Autoimmunerkrankungen wie Myasthenia gravis beschrieben. Der P. vulgaris beginnt meist umschrieben mit uncharakteristischen Läsionen an Mundschleimhaut (siehe auch S. 443; Abb. 20.3), Kopfhaut oder Intertrigines, später kommt es zu generalisiertem Befall des Integumentes und sämtlicher hautnaher Schleimhäute unter Einschluss des Laryngopharynx. Klinisch imponieren flächige, schmerzhaft-brennende, nässende Erosionen mit randwärts halskrausenartig zusammengeschobenen Epidermisfetzen, die hämorrhagisch verkrusten oder impetiginisieren. Intakte Epidermis lässt sich durch seitlichen Druck tangential abschieben (Nikolski-Zeichen). Im Verlauf treten auch schlaffe Blasen mit klarem Inhalt ohne weitere Entzündungszeichen auf. Die Abheilung erfolgt in der Regel narbenlos. Die Krankheit verläuft schubweise progredient und endet unbehandelt aufgrund von Flüssigkeits- und Elektrolytverlusten, Sepsis, Bronchopneumonien oder Kachexie häufig letal. Der P. vulgaris spricht gut auf immunsuppressive Therapien an.

Synonym

Pemphigus benignus familiaris chronicus „familial benign chronic pemphigus“ Dyskeratosis follicularis „Darier-White disease“ transiente akantholytische Dermatose benigne papulöse akantholytische Dermatose staphylogenes Lyell-Syndrom Dermatitis exfoliativa neonatorum Ritter von Rittershain subkorneale pustuläre Dermatose Sneddon-Wilkinson-Syndrom akut generalisierte exanthematische pustulöse Dermatitis akut generalisierte exanthematische Pustulosis akut generalisierte exanthematische pustulöse Dermatitis „general pustular drug rash“ 

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© Springer-Verlag Berlin Heidelberg 2003

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  • D. Metze

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