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Immunoglobulin A Dermatoses

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Clinical and Basic Immunodermatology

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Abstract

Immunoglobulin A (IgA) dermatoses represent a distinct but diverse category of immunologic skin diseases, including linear IgA bullous dermatosis, dermatitis herpetiformis, IgA subcorneal pemphigus, IgA intraepidermal pemphigus and IgA vasculitis (Henoch-Schönlein purpura).

The common findings that tie all of these disorders together are the cutaneous deposition of IgA at the histopathologic site of inflammation on direct immunofluorescence microscopy and a neutrophilic inflammatory infiltrate on hematoxylin and eosin staining of involved skin.

IgA is found in respiratory, gastrointestinal and genitourinary mucosal secretions, as well as human serum. The IgA dermatoses share activation of the IgA receptor, FcαR1, on neutrophils, which is also present on eosinophils and monocytes. This receptor activation starts an inflammatory cascade believed to be essential in these disorders that include: a neutrophilic response, endocytosis, antibody-dependent cell-mediated cytotoxicity, the respiratory burst, degranulation in the skin and subsequent tissue damage.

The main therapy for these disorders remains dapsone, a sulfone antibiotic, and its related medications, which are effective agents against neutrophil activity that include inhibition of adhesion, chemotaxis and myeloperoxidase production for the respiratory burst. Topical therapy consists mainly of high potency corticosteroids.

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Authors and Affiliations

  1. Department of Dermatology, University of Utah School of Medicine, 30 N. 1900 E, Suite 4A330, Salt Lake City, UT, 84132, USA

    Julia A. Curtis MD & John J. Zone MD

Authors
  1. Julia A. Curtis MD
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  2. John J. Zone MD
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Corresponding author

Correspondence to Julia A. Curtis MD .

Editor information

Editors and Affiliations

  1. School of Medicine, University of Maryland Baltimore, Baltimore, Maryland, USA

    Anthony A. Gaspari

  2. Department of Dermatology, University of Texas Health Science Center, Houston, Texas, USA

    Stephen K. Tyring

  3. Department of Dermatology and Immunology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA

    Daniel H. Kaplan

Questions

Questions

  1. 1.

    What are the common findings in the skin for all of the IgA dermatoses?

    1. A.

      Cutaneous deposition of IgA at the histopathologic site of inflammation on direct immunofluorescence microscopy and a neutrophilic inflammatory infiltrate on hematoxylin and eosin staining of involved skin

  2. 2.

    Where is IgA found in the body?

    1. A.

      IgA is found in respiratory, gastrointestinal and genitourinary mucosal secretions, as well as human serum

  3. 3.

    What is the name of the IgA receptor common to these disorders?

    1. A.

      FcαR1

  4. 4.

    What are some of the cutaneous findings in these conditions?

    1. A.

      Bullae, vesicles, papules and pustules

  5. 5.

    What is the main therapy for the IgA dermatoses?

    1. A.

      Dapsone

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Curtis, J.A., Zone, J.J. (2017). Immunoglobulin A Dermatoses. In: Gaspari, A., Tyring, S., Kaplan, D. (eds) Clinical and Basic Immunodermatology. Springer, Cham. https://doi.org/10.1007/978-3-319-29785-9_34

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