摘要目的 加深对乳房外Paget病(EMPD)临床和病理特点的认识,探讨棘层松解现象在EMPD中的临床和病理意义.方法 对北京协和医院皮肤科2010年6月至2016年10月间诊治的56例EMPD患者的临床和病理资料进行回顾性分析.根据是否伴发棘层松解现象,将患者分为棘层松解性乳房外Paget病(AEMPD)组和不伴棘层松解的乳房外Paget病(N-AEMPD)组.结果 AEMPD组患者22例,占39.3％.男女比例10:1,平均发病年龄为(64±8)岁,中位病程42个月(4～240个月).N-AEMPD组患者34例,占60.7％.男女比例10.3∶1,平均发病年龄为(64±10)岁,中位病程48个月(3～ 120个月).两组患者在男女比例、发病年龄和病程上的差异无统计学意义(均P＞0.05).AEMPD组和N-AEMPD组患者中真皮内重度炎症、肿瘤细胞间变和肿瘤细胞累及附属器上皮或真皮浸润的发生率分别为27.3％和17.6％ (P =0.600);13.6％和17.6％ (P =0.979);72.7％和23.5％ (P ＜0.001).Hailey-Hailey病型AEMPD 17例,占77.3％.接受手术且有明确随访资料者29例,其中AEMPD组12例,1例复发;N-AEMPD组17例,4例复发;两组患者复发率的差异无统计学意义(P =0.370).结论 EMPD中棘层松解现象常见,棘层松解与患者的性别、发病年龄和病程无关.EMPD中出现棘层松解可提示肿瘤细胞的侵袭性生长,但与真皮重度炎症和Paget细胞间变无关.Hailey-Hailey病型AEMPD最常见,应提高警惕,避免误诊.棘层松解与EMPD的术后复发无相关性.

abstractsObjective To identify the clinicopathological features of extramammary Paget's disease (EMPD) and investigate the clinical and histopathological significance of acantholysis in EMPD.Methods From June,2010 to October,2016,a total of 56 patients were diagnosed as EMPD in the Department of Dermatology,Peking Union Medical College Hospital.Clinical and histopathological data were retrieved from these patients' medical records and analyzed respectively.The cases were divided into two subgroups according to the histopathological pattern (with or without acantholysis):the acantholytic EMPD (AEMPD) group and the non-acantholytic EMPD (N-AEMPD) group.The clinicopathological data were compared and statistically analyzed between the two groups.Results The AEMPD group included 22 cases (39.3％),while the N-AEMPD group included 34 cases (60.7％).The male:female ratio,the age of onset,and the median duration of the disease were 10:1 vs 10.3:1,(64 ± 8) vs (64 ± 10) years,and 42 (4-240) months vs 48(3-120) months in the AEMPD and N-AEMPD groups,respectively,with no significant difference (all P ＞0.05).There was no significant difference in the severe dermal inflammation (27.3％ vs 17.6％,P =0.600) and cytologic anaplasia(13.6％ vsl7.6％,P =0.979) between the AEMPD and N-AEMPD groups.Adnexal involvement or dermal invasion (72.7％) was significantly more common in cases with AEMPD,compared to cases with N-AEMPD (23.5％,P ＜ 0.001).And 17 cases in the AEMPD group (77.3％) were Hailey-Hailey-disease-like subtype.The recurrence rate after surgical management (29 cases) showed no significant difference between the two groups (1/12 vs 4/17,P =0.370).Conclusions Acantholysis is common in EMPD.It is not associated with sex,the age of onset,and the duration of the disease.Acantholysis may indicate invasive growth of Paget's cells.Its occurrence has no association with severe dermal inflammation or cytologic anaplasia.Hailey-Hailey-disease-like subtype is the most common subtype in AEMPD,requiring careful consideration to avoid misdiagnosis.Postoperative recurrence is not associated with acantholysis in EMPD.