Pemphigus vegetans variant of IgA pemphigus, a variant of IgA pemphigus and other autoimmune blistering disorders

Michael M. Wolz, Michael J. Camilleri, Marian T. Mcevoy, Alison J. Bruce

Research output: Contribution to journalArticle

16 Scopus citations

Abstract

Pyodermatitis-pyostomatitis vegetans (PPV) constitutes an inflammatory mucocutaneous dermatosis that is associated with inflammatory bowel disease. Clinically, PPV appears as pustules on mucosal surfaces and as vegetating exudative plaques on intertriginous surfaces. It is typically a clinical diagnosis supported by histological findings. Microscopic findings include epidermal hyperplasia, focal acantholysis, and a dense mixed inflammatory infiltrate with intraepithelial and subepithelial eosinophilic microabscesses. In the recent literature, immunofluorescence has been thought to be negative in PPV or, if positive, an aberrant finding. Herein, we report 2 cases of PPV associated with inflammatory bowel disease, which display intercellular IgA deposits. Although these cases may represent isolated epiphenomena, it is possible that the paucity of PPV cases with immunofluorescent studies hitherto has led to an oversight of an interesting association between intercellular IgA and PPV.

Original languageEnglish (US)
Pages (from-to)e53-e56
JournalAmerican Journal of Dermatopathology
Volume35
Issue number3
DOIs
StatePublished - May 1 2013

Keywords

  • Immunodermatology
  • Inflammatory bowel disease
  • Pemphigoid
  • Pemphigus
  • Pyodermatitis-pyostomatitis vegetans

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Dermatology

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