Extramammary Paget disease (EMPD) is a rare intraepithelial adenocarcinoma. The current mainstay of treatment is wide local excision. We present the case of a 56-year-old woman with perianal EMPD that recurred 4 years after initial treatment with wide local excision with Mohs micrographic surgery tissue processing of marginal tissue. Upon recurrence with anal canal involvement, the patient was treated with a 16-week combination course of topical imiquimod and oral cimetidine. There is growing evidence to support both the use of topical imiquimod for the treatment of EMPD as well as the antioncogenic effects of oral cimetidine. We present this case of primary perianal EMPD to highlight an alternative treatment regimen for poor surgical candidates.
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