Extramammary Paget's disease (EMPD) is a disorder that frequently affects the anogenital region in the elderly. Clinically, it presents a well circumscribed, reddish brown plaque. Histopathologically, the lesion is characterized by large pale "Paget's cells" usually restricted to the epidermis. In general, approximately 20% of affected individuals may develop either a subjacent eccrine or apocrine carcinoma or an internal malignancy, mainly rectal, vaginal, or bladder carcinoma. A high recurrence rate has been reported after excision. We present a case of extramammary Paget's disease with intradermal tumor, which entirely consists of Paget cells and was connected to intraepidermal Pagetoid proliferation by several columns.
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