title: Pyostomatitis vegetans (PSV)-pyodermatitis vegetans (PDV): A clinicopathologic study of 7 cases at a tertiary referral center.
abstract: BACKGROUND
  Pyostomatitis vegetans (PSV)-pyodermatitis vegetans (PDV) is a rare inflammatory mucocutaneous disease associated with inflammatory bowel disease.
  
  
  OBJECTIVE
  We sought to evaluate the clinicopathologic findings of PSV-PDV in a series of 7 patients.
  
  
  METHODS
  We conducted a retrospective review of all cases of PSV-PDV at the Mayo Clinic from 1995 to 2014.
  
  
  RESULTS
  Seven patients with PSV-PDV were included, and all had inflammatory bowel disease. Three had Crohn's disease and 4 had ulcerative colitis. Three patients had peripheral blood eosinophilia. Two had concomitant pyoderma gangrenosum in which pyoderma gangrenosum lesions were recalcitrant to therapy. Primary sclerosing cholangitis was seen in 3 patients. Two patients had direct and 3 had indirect immunofluorescence findings. Tissue eosinophilia was seen in the majority of mucosal and cutaneous lesions.
  
  
  CONCLUSIONS
  Limited sample size and retrospective study design are limitations.
  
  
  CONCLUSIONS
  PSV-PDV is associated with inflammatory bowel disease and primary sclerosing cholangitis and may precede gastrointestinal symptoms. Immunofluorescence findings in select PSV-PDV cases may indicate possible overlap with autoimmune bullous disease. Tissue eosinophilia may be helpful in distinguishing PSV-PDV from pyoderma gangrenosum. Strict control of bowel disease and close monitoring of patients with subclinical disease is warranted.
authors.affiliation: Mayo Medical School, College of Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Leon G
authors.initials: LG
authors.lastname: Clark
authors.affiliation: Department of Dermatology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Stanislav N
authors.initials: SN
authors.lastname: Tolkachjov
authors.affiliation: Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Division of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Alina G
authors.initials: AG
authors.lastname: Bridges
authors.affiliation: Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Division of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota. Electronic address: camilleri.michael1@mayo.edu.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Camilleri
mesh_terms: D000328:Adult; D017677:Age Distribution; D001707:Biopsy, Needle; D015897:Comorbidity; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D015212:Inflammatory Bowel Diseases / Q000175:diagnosis / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011711:Pyoderma / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology*; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D012720:Severity of Illness Index; D017678:Sex Distribution; D013280:Stomatitis / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology*; D062606:Tertiary Care Centers; D055815:Young Adult
keywords: Crohn's disease; inflammatory bowel disease; pemphigus; primary sclerosing cholangitis; pyoderma gangrenosum; pyodermatitis; pyostomatitis; ulcerative colitis; vegetans