Introduction Intraepithelial Extramammary Paget’s disease (EMPD) of the vulva is a rare neoplasm with high rates of recurrence. EMPD in a split-thickness skin graft, is associated with retrodissemination or spread of the disease within the skin via lymphatics and vessels creating tissue bridges between sites of involvement.
We present a case of an 81-year-old Asian female, with complaints of vulvar pruritus and lesion at the left inguinal. Enlargement of the lesion prompted a vulvar punch biopsy which showed Paget’s disease. Wide local excision with split-thickness skin grafting was performed. One-year post-operation, vulvar lesions on the split-thickness graft were noted. Biopsy showed Extramammary Paget’s Disease recurrence. Patient underwent repeat wide local excision with frozen section, and split-thickness skin grafting. After 6 months post re-excision, patient noted vulvar lesions and repeat biopsy showed Extramammary Paget’s Disease recurrence. Due to the proximity of the lesion to the sphincter and need for a colostomy, the patient did not consent for re-excision. Imiquimod 5% was chosen as the mode of treatment.
Conclusion Retrodissemination is hypothesized as the etiology of Paget’s spread in a split-thickness skin graft. Surgical challenges include removing the disease that may not be visible and minimizing morbidity from radical surgery. Imiquimod 5% can be used in recurrences. Despite the advances in the knowledge of EMPD of the vulva, the high rate of recurrent disease remains a challenge for optimal management and would require frequent and long-term follow-up.
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