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Bullöse Autoimmundermatosen

  • Christian Sadik
  • Detlef Zillikens
  • Michael Hertl
Chapter
Part of the Springer Reference Medizin book series (SRM)

Zusammenfassung

Bullöse Autoimmundermatosen sind Folge einer Autoantikörper-getriebenen Immunantwort gegen Strukturproteine der Haut. Die Ursache der Autoantikörperbildung ist weitgehend unbekannt. Es werden drei Erkrankungsgruppen bullöser Autoimmundermatosen unterschieden, die sich in Pathogenese und klinischem Bild deutlich unterscheiden, obwohl sie sich alle drei typischerweise mit entzündlichen Blasen der Haut manifestieren: Die Pemphigus- und Pemphigoid-Erkrankungen sowie die Dermatitis herpertiformis Duhring. Bei Pemphigus-Erkrankungen findet eine Autoantikörper-Bildung gegen desmosomale Adhäsionsproteine statt, die den epidermalen Zell-Zell-Kontakt vermitteln. Dies führt zu einer intraepidermalen Blasenbildung. Im Gegensatz dazu sind bei den Pemphigoiden die Blasen subepidermal gelegen und werden durch Autoantikörper gegen hemidesmosomale Strukturproteine oder Verankerungsfibrillen der dermo-epidermalen Junktionszone mit nachfolgender Rekrutierung von Granulozyten in die Haut verursacht.

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Copyright information

© Springer-Verlag GmbH Deutschland, ein Teil von Springer Nature 2018

Authors and Affiliations

  • Christian Sadik
    • 1
  • Detlef Zillikens
    • 1
  • Michael Hertl
    • 2
  1. 1.Klinik für Dermatologie, Allergologie und VenerologieUniversität zu LübeckLübeckDeutschland
  2. 2.Klinik für Dermatologie und AllergologieUniversitätsklinikum MarburgMarburgDeutschland

Section editors and affiliations

  • Michael Hertl
    • 1
  1. 1.Zentrum für HautkrankheitenMarburgGermany

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