Vulvar extramammary Paget disease

Vulvar extramammary Paget disease (EMPD) is a rare intraepithelial neoplasm that presents as eczematous, pruritic plaques of the perineum. Most vulvar EMPD is intraepithelial and noninvasive, but 10%–15% of cases are invasive. Metastatic disease is rare but is associated with poor prognosis. Work-up of vulvar EMPD includes vulvar biopsy and screening for secondary malignancies. Further imaging can be performed when metastatic disease is suspected. Primary treatment for localized vulvar EMPD is surgical resection. However, local recurrence is common, and repeat excisions can be associated with significant morbidity. Noninvasive treatments including topical imiquimod, CO2 laser ablation, radiation, photodynamic therapy, and systemic therapy, including cytotoxic chemotherapy and trastuzumab, are used with varying efficacy to treat metastatic, recurrent, or nonoperable disease.