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Ichthyosen

  • Vinzenz Oji
Chapter
Part of the Springer Reference Medizin book series (SRM)

Zusammenfassung

Hereditäre Ichthyosen sind erbliche Verhornungsstörungen, die sich auf die gesamte Haut beziehen und durch Verdickung der Hornhaut und Schuppung gekennzeichnet sind. Pathophysiologisch gesehen handelt es sich primär um Störungen der epidermalen Barriere. Die Vielzahl der äußerst seltenen Erkrankungen zu überblicken, ist eine Herausforderung: Die Klassifikation erfolgt auf klinisch-genetischer Basis und nach nichtsyndromalen oder syndromalen Formen. Die diagnostische Abgrenzung der Ichthyosis vulgaris und der X-chromosomal-rezessiven Ichthyose von den seltenen kongenitalen Ichthyosen ist von großer praktischer Bedeutung. Zu unterscheiden sind zudem erworbene ichthyosiforme Hautzustände. In diesem Kapitel werden die faszinierenden einzelnen genetischen Störungen erklärt. Zur Schärfung des klinischen Blicks ist es mit ausführlichem Bildmaterial unterlegt. Die Therapiehinweise folgen der Maxime, dass mittels symptomatischer Therapiemaßnahmen viel für die Patienten erreicht werden kann.

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Copyright information

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

Authors and Affiliations

  1. 1.Klinik und Poliklinik für HautkrankheitenUniversitätsklinikum MünsterMünsterDeutschland
  2. 2.Hautarztpraxis am BuddenturmMünsterDeutschland

Section editors and affiliations

  • Michael Hertl
    • 1
  1. 1.Klinik für Dermatologie und Allergologie, Universitätsklinikum MarburgMarburgDeutschland

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