2005 Volume 115 Issue 12 Pages 1791-1795
PD-PSV is a rare inflammatory disease of unknown pathogenesis, characterized by pustular and vegetating mucocutaneous lesions. Its association with inflammatory bowel disease is well established. We report the case of a 26-year-old man who had suffered from ulcerative colitis for nine years. Three years earlier, multiple pustules and erosions appeared on his gingivae, and, in 2000, exudative vesiculopustular plaques appeared on his scalp and inguinal regions. A skin biopsy showed microabscesses with neutrophils and eosinophils within the hyperplastic epidermis. Direct immunofluorescence studies revealed negative results, and anti-desmoglein 1 and 3 antibodies were negative. This case suggests that PD-PSV is an entity distinct from pemphigus vegetans.