Primary invasive triple extramammary Paget's disease with regional lymph node metastasis: A case report and review of the literature

Extramammary Paget's disease (EMPD) is a rare intraepidermal carcinoma and predominantly involves apocrine gland-bearing areas, such as anogenital regions and axillae. EMPD usually involves a solitary area and, less often, two areas in the same patient (double EMPD). The simultaneous involvement of bilateral axillae and anogenital region, called triple extramammary Paget's disease (TEPD), is an extremely rare subgroup of diseases that has been reported mostly from studies conducted in Japan. Because of its rarity, the clinical course, pathology/immunohistochemical staining features, and prognosis of TEPD are still unclear. Herein, to our knowledge, we present the first case of primary invasive TEPD with regional lymph node metastasis in Taiwan, and review the literature.