Pathogenese bullöser Autoimmunkrankheiten

  • Klaus Wolff
Conference paper
Part of the Fortschritte der praktischen Dermatologie und Venerologie book series (DERMATOLOGIE, volume 14)

Zusammenfassung

Vor mehr als 25 Jahren beschrieben Jordon und Beutner [2] zirkulierende Autoantikörper bei Pem phigus vulgaris und bullösem Pemphigoid, denen aufgrund ihrer In-vivo-Fixation an Epidermis bzw. Basalmembranzone und damit dem Ort der für die se Krankheiten typischen Läsionen eine pathogenetische Relevanz zuzukommen schien. Es wurde da mit eine Entwicklung in Gang gesetzt, die heute zu der Erkenntnis geführt hat, daß die meisten der be kannten erworbenen blasenbildenden Dermatosen antikörpermediierte, organspezifische Autoimmun krankheiten darstellen [32]. Der Verlust der Gewebskohärenz und die daraus resultierende Blasen bildung beruht bei diesen Krankheiten auf einem Verlust der Adhäsionskräfte entweder zwischen den Epidermalzellen oder zwischen Epidermalzellen und Basalmembran oder zwischen Basalmembran und diese in der Dermis verankernden Gewebskomponenten. Da die meisten der dafür verant wortlichen molekularen Strukturen bekannt sind, verstehen wir nun, warum z. B. die akantholytische Blasenbildung bei Pemphigus vulgaris suprabasal in der Epidermis und die Spaltbildung bei Epidermolysis bullosa acquisita im obersten Korium erfol gen. Wir können also heute zwischen klinischem Bild und der molekularen Zielstruktur des Auto immunprozesses einen Bogen spannen und haben damit ein wesentlich klareres Verständnis der Pa thogenese dieser Erkrankungen, als das noch vor wenigen Jahren der Fall war.

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© Springer-Verlag Berlin Heidelberg 1995

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  • Klaus Wolff

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