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

Am J Dermatopathol. 2013 May;35(3):e53-6. doi: 10.1097/DAD.0b013e318278d419.

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.

Publication types

  • Case Reports

MeSH terms

  • Autoimmunity* / drug effects
  • Biopsy
  • Colitis, Ulcerative / complications
  • Colitis, Ulcerative / drug therapy
  • Colitis, Ulcerative / immunology*
  • Crohn Disease / complications
  • Crohn Disease / drug therapy
  • Crohn Disease / immunology*
  • Humans
  • Immunoglobulin A / analysis*
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Microscopy, Fluorescence
  • Middle Aged
  • Mouth Mucosa / drug effects
  • Mouth Mucosa / immunology*
  • Mouth Mucosa / pathology
  • Pemphigus / classification
  • Pemphigus / drug therapy
  • Pemphigus / immunology*
  • Pemphigus / pathology
  • Skin / drug effects
  • Skin / immunology*
  • Skin / pathology
  • Treatment Outcome
  • Young Adult

Substances

  • Immunoglobulin A
  • Immunosuppressive Agents