Extramammary Paget's disease (EMPD) is a rare neoplastic condition that commonly affects the anogenital area in the elderly. Owing to its low incidence, limited data regarding EMPD's diagnosis and treatment have been available. This review article aims to explore the current knowledge of EMPD to improve the management of this disease. Areas covered: This review outlines the diagnosis and management of EMPD. Articles on this issue that had been published in PubMed were identified and surveyed. We provide an overview of the reported studies, focusing on the recent advances in this field. Expert commentary: A new TNM staging system specific for EMPD has been proposed in Japan; the T category was classified by tumor thickness and lymphovascular invasion, the N category by the number of metastatic lymph nodes, and the M category by systemic metastases. As new diagnostic tools for EMPD, dermoscopy and reflectance confocal microscopy have emerged. Recent reports about Mohs micrographic surgery, mapping biopsy, radiation therapy, photodynamic therapy, topical imiquimod, conventional chemotherapy, and targeted therapy are also discussed. Despite the increasing publications of EMPD, limited information on this condition is available and the accumulation of more data is required.
Keywords: Chemotherapy; TNM stage; dermoscopy; extramammary Paget’s disease; imiquimod; mapping biopsy; prognostic factor; surgery; targeted therapy; treatment; tumor thickness.