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Subcorneal and Intraepidermal Immunobullous Dermatoses

  • Monika Bowszyc-Dmochowska
  • Mai P. Hoang
  • Marian DmochowskiEmail author
Chapter
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Abstract

The diseases described in this chapter are the intraepidermal bullous dermatoses exhibiting acantholysis as the main and common histologic feature. The first group are pemphiguses or acquired autoimmune bullous diseases with autoantibodies directed to desmosomal proteins mostly desmogleins 1 and 3 and rarely other keratinocytic proteins. The two main subgroups are pemphigus foliaceus with subcorneal blisters and desmoglein 1 autoantibodies and pemphigus vulgaris with suprabasilar acantholysis and anti-desmoglein 3 and less commonly anti-desmoglein 1 autoantibodies. The following seven pemphigus types including vegetans, herpetiformis, erythematosus, paraneoplastic, drug-induced, IgA, and IgA/IgG are variants of the main subgroups. In the second group of disorders with acantholysis and Hailey-Hailey and Darier diseases, genetic defect is responsible for desmosomal instability with or without abnormal keratinization. The last entity, Grover disease, is acquired with late-onset transient acantholytic dermatosis that histopathologically resembles autoimmune or genetic acantholytic disorders without circulating autoantibodies and known genetic background.

Keywords

Acantholysis Dyskeratosis Pemphigus Grover disease Benign familial pemphigus Autoimmune disorders Histology Desmogleins Desmocollins 

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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Monika Bowszyc-Dmochowska
    • 1
  • Mai P. Hoang
    • 2
  • Marian Dmochowski
    • 3
    Email author
  1. 1.Cutaneous Histopathology and Immunopathology Section, Department of DermatologyPoznan University of Medical SciencesPoznanPoland
  2. 2.Department of PathologyMassachusetts General Hospital and Harvard Medical SchoolBostonUSA
  3. 3.Autoimmune Blistering Dermatoses Section, Department of DermatologyPoznan University of Medical SciencesPoznanPoland

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