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 Bruce

Research output: Contribution to journalArticle

15 Citations (Scopus)

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)
JournalAmerican Journal of Dermatopathology
Volume35
Issue number3
DOIs
StatePublished - May 1 2013

Fingerprint

Pemphigus
Immunoglobulin A
Inflammatory Bowel Diseases
Acantholysis
Skin Diseases
Hyperplasia
Fluorescent Antibody Technique
vegetan

Keywords

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

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Dermatology

Cite this

Pemphigus vegetans variant of IgA pemphigus, a variant of IgA pemphigus and other autoimmune blistering disorders. / Wolz, Michael M.; Camilleri, Michael J.; Mcevoy, Marian T.; Bruce, Alison.

In: American Journal of Dermatopathology, Vol. 35, No. 3, 01.05.2013.

Research output: Contribution to journalArticle

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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.",
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