Pyostomatitis vegetans (PSV)-pyodermatitis vegetans (PDV): A clinicopathologic study of 7 cases at a tertiary referral center

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• 作者：Leon G. Clark ; BA^a ; Stanislav N. Tolkachjov ; MD^b ; Alina G. Bridges ; DO^b ; ^c ; Michael J. Camilleri ; MD^b ; ^c ; ^{camilleri.michael1@mayo.edu" class="auth_mail" title="E-mail the corresponding author}
• 关键词：Crohn's disease ; inflammatory bowel disease ; pemphigus ; primary sclerosing cholangitis ; pyoderma gangrenosum ; pyodermatitis ; pyostomatitis ; ulcerative colitis ; vegetans
• 刊名：Journal of the American Academy of Dermatology
• 出版年：2016
• 出版时间：September 2016
• 年：2016
• 卷：75
• 期：3
• 页码：578-584
• 全文大小：1235 K

文摘

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.