TY - JOUR
T1 - Pemphigus vegetans variant of IgA pemphigus, a variant of IgA pemphigus and other autoimmune blistering disorders
AU - Wolz, Michael M.
AU - Camilleri, Michael J.
AU - Mcevoy, Marian T.
AU - Bruce, Alison J.
PY - 2013/5
Y1 - 2013/5
N2 - 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.
AB - 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.
KW - Immunodermatology
KW - Inflammatory bowel disease
KW - Pemphigoid
KW - Pemphigus
KW - Pyodermatitis-pyostomatitis vegetans
UR - http://www.scopus.com/inward/record.url?scp=84876700378&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84876700378&partnerID=8YFLogxK
U2 - 10.1097/DAD.0b013e318278d419
DO - 10.1097/DAD.0b013e318278d419
M3 - Article
C2 - 23221471
AN - SCOPUS:84876700378
SN - 0193-1091
VL - 35
SP - e53-e56
JO - American Journal of Dermatopathology
JF - American Journal of Dermatopathology
IS - 3
ER -