Bahsas Zaky Osama, Puckett Yana
SOH-IVSS
Orlando Health Cancer Institute, Orlando, Florida
Extramammary Paget disease (EMPD) is a rare cutaneous adenocarcinoma that primarily affects older adults, typically those between 50 and 80 years of age. Data from the Surveillance, Epidemiology, and End Results (SEER) program indicate that the incidence of EMPD is highest among Asian men, followed by Caucasians and African Americans. Approximately 10% to 30% of patients have an associated internal malignancy, most commonly involving the colon, breast, or genitourinary tract. Several aspects of EMPD remain controversial, including the true prevalence of concurrent vulvar adenocarcinoma or invasive EMPD, its association with regional or distant malignancies, and recurrence rates following surgical excision. The disease usually arises in areas with a high density of apocrine glands, most frequently the vulva, but also the perineal, scrotal, perianal, and penile regions. Treatment typically involves surgical excision, but may also include radiotherapy, photodynamic therapy, systemic or topical chemotherapy, and, in some cases, targeted therapy for metastatic disease.[3] Because EMPD often mimics benign infectious or inflammatory dermatoses, diagnosis is frequently delayed, leading to increased morbidity.
乳房外佩吉特病（EMPD）是一种罕见的皮肤病，常出现在大汗腺丰富的区域，最常见于外阴，不过会阴、阴囊、肛周和阴茎皮肤也可能受累。病变在临床上表现为边界清晰的红斑性斑块，可能会出现糜烂、溃疡、鳞屑或湿疹样改变。乳房外佩吉特病以女性居多，通常发生在60至80岁之间。专业人士在乳房外佩吉特病的许多方面存在分歧，例如，同时存在外阴腺癌或侵袭性乳房外佩吉特病的患病率、与区域及远处癌症的关联以及手术切除后的复发率。早期识别至关重要，因为诊断常常延迟，且相关侵袭性疾病的发生率很高。
