Ishiguro Akihiro, Iwashita Nobuhiko, Abe Michihiro, Ogawa Akina, Takeo Tomohiro, Watanabe Daisuke
Dermatology, Aichi Medical University School of Medicine, Nagakute, Japan.
Case Rep Dermatol. 2024 Jun 17;16(1):149-155. doi: 10.1159/000538675. eCollection 2024 Jan-Dec.
Extramammary Paget's disease (EMPD) is a rare skin cancer that tends to be multicentric, with quadruple EMPD cases being scarcely reported.
We report the case of an 81-year-old man with heterochronous quadruple EMPD. Twelve years after total resection of vulvar EMPD, the patient developed erythematous lesions on the resection margin in the lower abdomen, umbilical region, and both axillae. Histological examination revealed that all lesions were in situ EMPD.
We reviewed six reported cases of quadruple EMPD with respect to race, sex, site, recurrence, time to recurrence, serum carcinoembryonic antigen, and depth. All patients were elderly Japanese males. In all but one case, the lesions were located in the apocrine region, which is a common site in such as the genital and axillary areas. Our case was the only heterochronous quadruple EMPD. The lesions were limited to the epidermis; therefore, they were unlikely to cause metastasis. It has been reported that the therapeutic effects of imiquimod can be expected in in situ EMPD. Therefore, quadruple EMPD may be a good indication of treatment option.
EMPD is a disease whose pathogenesis is not yet clear; however, it is hoped that the origin and aetiology of EMPD will be elucidated from the clinical features of multiple EMPD in the future.
乳腺外佩吉特病（EMPD）是一种罕见的皮肤癌，往往呈多中心性，四发性EMPD病例鲜有报道。
我们报告了一例81岁患有异时性四发性EMPD的男性患者。在外阴EMPD全切术后12年，患者在下腹部、脐部和双侧腋窝的手术切缘处出现红斑性病变。组织学检查显示所有病变均为原位EMPD。
我们回顾了6例已报道的四发性EMPD病例，涉及种族、性别、部位、复发情况、复发时间、血清癌胚抗原及深度。所有患者均为老年日本男性。除1例病例外，所有病例的病变均位于顶泌汗腺分布区，这是生殖器和腋窝等部位的常见区域。我们的病例是唯一的异时性四发性EMPD。病变局限于表皮，因此不太可能发生转移。据报道，咪喹莫特对原位EMPD可能有治疗效果。因此，四发性EMPD可能是一个很好的治疗选择指征。
EMPD是一种发病机制尚不清楚的疾病；然而，希望未来能从多发性EMPD的临床特征中阐明EMPD的起源和病因。
