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Desmosomal Proteins as Autoantigens in Pemphigus

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Blistering Diseases

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Abstract

Pemphigus is a group of rare autoimmune blistering skin diseases mediated by autoantibodies against desmosomal members of the cadherin superfamily. There are several variants of pemphigus, each with unique clinical, histological, and immunologic features. Interestingly, when different desmosomal proteins are targeted by the autoimmune response, different clinical and histological features are seen. In this chapter we review the key intercellular junctions and proteins that mediate keratinocyte adhesion, the pemphigus group disease variants, and the specific antigenic protein(s) targeted in each and conclude with a discussion of pathogenic mechanisms that may explain how autoantibody binding these protein targets induces acantholysis and clinical disease.

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Authors and Affiliations

  1. Department of Microbiology and Immunology/Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA

    Flor Evangelista BS

  2. Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA

    Donna A. Culton MD, PhD & Luis A. Diaz MD

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  1. Flor Evangelista BS
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  2. Donna A. Culton MD, PhD
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  3. Luis A. Diaz MD
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Correspondence to Donna A. Culton MD, PhD .

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Editors and Affiliations

  1. Department of Dermatology, St George Hospital, University of New South Wales, Sydney, New South Wales, Australia

    Dédée F. Murrell

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Evangelista, F., Culton, D.A., Diaz, L.A. (2015). Desmosomal Proteins as Autoantigens in Pemphigus. In: Murrell, D. (eds) Blistering Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45698-9_5

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  • DOI: https://doi.org/10.1007/978-3-662-45698-9_5

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