M

rd nE xtramammary Paget’s disease (EMPD) is an uncommon malignancy that usually affects the genital skin, and less commonly the axilla, in elderly patients. It is thought to be an apocrine tumor, which arises within the epidermis from native stem cells that differentiate toward glandular cells.1 EMPD is often undiagnosed for years as it can mimic contact or irritant dermatitis, seborrheic dermatitis, tinea cruris, candidiasis, inverse psoriasis, or Bowen’s disease. There are reports of EMPD arising in the eyelids,2 ear canal,3 and the umbilicus.4 Standard surgical management procedures with wide local excision, vulvectomy, or abdominoperineal resec-tion can carry significant morbidity and deformity. The recurrence rates with these modalities are relatively high (up to 60%).5 This is likely because of the characteristic microscopic extension of EMPD beyond the clinically visible margins.1 The use of Mohs micrographic surgery (MMS) in the management of EMPD has been documented in the medical literature.6-11 However, there are no large cohorts evaluating the efficacy of MMS in the management of EMPD. MMS differs from routine excision with histologic margin examination by providing intraoperative microscopic evaluation of 100 % of the tissue margin. The technique allows for the microscopically guided excision of tumors and preservation of normal tissue. In contrast, routine frozen sections, done without Mohs technique of enface sectioning, sample less than 0.1 % of the surgical margin. To examine the entire margin of 1 cm of submitted specimen by serial vertical section-ing, as many as 1500 sections (7-m thick) would be required.12 The paucity of data from a large cohort, and our successful experience with treating EMPD with MMS, led us to review the cases performed during a period of 20 years