Intraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis

J Am Acad Dermatol. 2021 Jun;84(6):1507-1519. doi: 10.1016/j.jaad.2020.11.075. Epub 2021 Mar 5.

Abstract

Intraepithelial autoimmune blistering dermatoses are a rare group of skin disorders characterized by the intraepithelial disruption of intercellular connections through the action of autoantibodies. The first article in this continuing medical education series explores the background, epidemiology, clinical features, and diagnostic criteria of each of the major intraepithelial autoimmune blistering dermatoses, including pemphigus foliaceus, pemphigus erythematosus, pemphigus herpetiformis, fogo selvagem, pemphigus vulgaris, pemphigus vegetans, drug-induced pemphigus, IgA pemphigus, IgG/IgA pemphigus, and paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome.

Keywords: IgA pemphigus; IgA/IgG pemphigus; drug-induced pemphigus; fogo selvagem; paraneoplastic autoimmune multiorgan syndrome; paraneoplastic pemphigus; pemphigus erythematosus; pemphigus foliaceus; pemphigus herpetiformis; pemphigus vegetans; pemphigus vulgaris.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / epidemiology
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / pathology
  • Diagnosis, Differential
  • Humans
  • Skin / immunology
  • Skin / pathology*
  • Skin Diseases, Vesiculobullous / diagnosis*
  • Skin Diseases, Vesiculobullous / epidemiology
  • Skin Diseases, Vesiculobullous / immunology
  • Skin Diseases, Vesiculobullous / pathology