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Subcorneal and Intraepidermal Immunobullous Dermatoses

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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.

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Authors and Affiliations

  1. Cutaneous Histopathology and Immunopathology Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland

    Monika Bowszyc-Dmochowska

  2. Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Mai P. Hoang

  3. Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland

    Marian Dmochowski

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  1. Monika Bowszyc-Dmochowska
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Correspondence to Marian Dmochowski .

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  1. Professor of Pathology, Harvard Medical School, Director of Dermatopathology, Massachusetts General Hospital, Boston, MA, USA

    Mai P. Hoang

  2. Professor of Pathology and Dermatology, Director, Dermatopathology Unit, Duke University Medical Center, Durham, NC, USA

    Maria Angelica Selim

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Bowszyc-Dmochowska, M., Hoang, M.P., Dmochowski, M. (2020). Subcorneal and Intraepidermal Immunobullous Dermatoses. In: Hoang, M., Selim, M. (eds) Hospital-Based Dermatopathology. Springer, Cham. https://doi.org/10.1007/978-3-030-35820-4_9

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