Png J C, Tung K H, Wong Y E, Mokal N, Rajagopalan R, Chong P Y
Department of Surgery, National University Hospital, Singapore.
Ann Acad Med Singap. 1995 Jul;24(4):636-9.
Extramammary Paget's disease (FMPD) is a rare skin disease with a tendency to recur even in the face of adequate excision. It was first suggested by Weiner in 1937 to be a carcinoma of the apocrine sweat gland with intraepithelial excision. Thus, most cases of EMPD occur in the perianal, perigenital and axilla regions where sweat glands abound, with scrotal EMPD being the commonest. EMPD can occur in both benign or malignant form and is often confused with chronic dermatitis. Diagnosis can only be confirmed by an excision biopsy. The long-term survival in patients without an underlying carcinoma is good. However, EMPD associated with an underlying adnexal carcinoma tends to be more aggressive and the prognosis is poor in such cases. EMPD is also associated with other malignancies such as prostatic, rectal, cervical, breast, bladder and skin carcinomas. Incidences of up to 40% have been reported. Thus, patients with EMPD should be thoroughly screened for associated primary growths. The treatment of EMPD is essentially by surgical excision with clear margins. The options available for a clear margin include intraoperative frozen section,Moh's surgery and paraffin section with delayed re-excision. In this report, we present three cases of EMPD with markedly different outcomes and a review of the literature.
乳房外佩吉特病（EMPD）是一种罕见的皮肤病，即使在充分切除后仍有复发倾向。1937年，维纳首次提出它是一种伴上皮内切除的顶泌汗腺癌。因此，大多数EMPD病例发生在汗腺丰富的肛周、生殖器周围和腋窝区域，其中阴囊EMPD最为常见。EMPD可呈良性或恶性形式，常与慢性皮炎相混淆。只有通过切除活检才能确诊。无潜在癌的患者长期生存率良好。然而，与潜在附件癌相关的EMPD往往更具侵袭性，此类病例的预后较差。EMPD还与其他恶性肿瘤相关，如前列腺癌、直肠癌、宫颈癌、乳腺癌、膀胱癌和皮肤癌。据报道，其发生率高达40%。因此，应对EMPD患者进行全面筛查以寻找相关的原发肿瘤。EMPD的治疗主要是进行切缘清晰的手术切除。实现切缘清晰的方法包括术中冰冻切片、莫氏手术以及石蜡切片并延迟再次切除。在本报告中，我们展示了3例结局明显不同的EMPD病例并对文献进行了综述。
