Leon G Clark1, Stanislav N Tolkachjov2, Alina G Bridges3, Michael J Camilleri4. 1. Mayo Medical School, College of Medicine, Mayo Clinic, Rochester, Minnesota. 2. Department of Dermatology, Mayo Clinic, Rochester, Minnesota. 3. Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Division of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota. 4. Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Division of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota. Electronic address: camilleri.michael1@mayo.edu.
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. LIMITATIONS: 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.
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. LIMITATIONS: 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.