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

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.

• Authors: Michael M Wolz, Michael J Camilleri, Marian T McEvoy, Alison J Bruce
• Journal: The American Journal of dermatopathology (Am J Dermatopathol) Vol. 35 Issue 3 Pg. e53-6 (May 2013) ISSN: 1533-0311 [Electronic] United States
• PMID: 23221471 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunoglobulin A
• Immunosuppressive Agents

Topics

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