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Autoimmune Bullous Diseases

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Braun-Falco´s Dermatology

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Abstract

Bullous autoimmune dermatoses are the result of an autoantibody-driven immune response against structural proteins of the skin. The cause of autoantibody formation is largely unknown. Three disease groups of bullous autoimmune dermatoses are distinguished, which differ clearly in pathogenesis and clinical picture, although they all typically manifest with inflammatory blisters of the skin: pemphigus and pemphigoid diseases, as well as dermatitis herpetiformis (Duhring’s disease). In pemphigus diseases, autoantibodies are formed against desmosomal adhesion proteins that mediate epidermal cell–cell contact. This leads to intraepidermal blister formation. In contrast, in pemphigoid diseases the blisters are subepidermal and are caused by autoantibodies against hemidesmosomal structural proteins or anchoring fibrils of the dermo-epidermal junction zone with subsequent recruitment of granulocytes into the skin.

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Authors and Affiliations

  1. Department of Dermatology, Allergology and Venerology, University of Lübeck, Lübeck, Germany

    Christian Sadik & Detlef Zillikens

  2. Department of Dermatology and Allergology, Philipps University Marburg, Marburg, Germany

    Michael Hertl

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  1. Christian Sadik
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  2. Detlef Zillikens
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  3. Michael Hertl
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Correspondence to Christian Sadik .

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Editors and Affiliations

  1. Department of Dermatology, University of Munich, Munich, Germany

    Gerd Plewig

  2. Department of Dermatology, University of Munich, Munich, Germany

    Lars French

  3. Department of Dermatology, University of Munich, Munich, Germany

    Thomas Ruzicka

  4. Department of Dermatology, Venerology and Allergology, Frankfurt University Hospital, Frankfurt, Germany

    Roland Kaufmann

  5. Clinic for Dermatology and Allergology, University of Marburg, Marburg, Germany

    Michael Hertl

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  1. Department of Dermatology and Allergology, Philipps University Marburg, Marburg, Germany

    Michael Hertl

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Sadik, C., Zillikens, D., Hertl, M. (2021). Autoimmune Bullous Diseases. In: Plewig, G., French, L., Ruzicka, T., Kaufmann, R., Hertl, M. (eds) Braun-Falco´s Dermatology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-58713-3_49-2

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  1. Latest

    Autoimmune Bullous Diseases
    Published:
    02 September 2021

    DOI: https://doi.org/10.1007/978-3-662-58713-3_49-2

  2. Original

    Autoimmune Bullous Diseases
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    29 July 2020

    DOI: https://doi.org/10.1007/978-3-662-58713-3_49-1

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