■乳房外佩吉特氏病(EMPD)是一种罕见的皮肤癌,往往是多中心的,几乎没有报告4例EMPD病例。
■我们报告了一例81岁男性非同步四重EMPD。外阴EMPD全切除12年后,患者在下腹部切除边缘出现红斑性病变,脐带区,和两个腋窝。组织学检查显示所有病变均为原位EMPD。
■我们回顾了六例报道的关于种族的四重EMPD病例,性别,site,复发,复发的时间,血清癌胚抗原,和深度。所有患者均为老年日本男性。除了一种情况,病变位于顶流区域,这是一个常见的部位,如生殖器和腋窝区域。我们的案例是唯一的非同步四重EMPD。病变仅限于表皮;因此,它们不太可能引起转移。据报道,咪喹莫特的治疗效果可以在原位EMPD中预期。因此,四联EMPD可能是治疗选择的良好指征。
■EMPD是一种发病机制尚不清楚的疾病;然而,希望将来从多个EMPD的临床特征中阐明EMPD的起源和病因。
UNASSIGNED: Extramammary Paget\'s disease (EMPD) is a rare skin cancer that tends to be multicentric, with quadruple EMPD cases being scarcely reported.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.