摘要目的 增加对棘层松解性乳房Paget病(AMPD)临床特征和组织病理学特点的认识.方法 回顾性分析北京协和医院皮肤科在2010年1月至2016年10月间诊治的28例AMPD患者的临床和病理资料.结果 28例AMPD患者均为女性,发病年龄24~78岁,平均发病年龄(51±15)岁,中位病程10.5个月(病程范围3个月~2年).所有患者均单侧乳头和(或)乳晕发病,左侧比右侧比例为1.55∶1.组织病理特征:患者均出现棘层松解模式,此外合并出现经典模式14例,占50.0%;合并上部巢状模式3例,占10.7%;合并芽蕾状模式2例,占7.1%;合并高巢团模式1例,占3.6%;合并全层模式1例,占3.6%. AMPD患者中Hailey-Hailey病型棘层松解占64.3%(18/28),寻常型天疱疮型棘层松解占25.0%(7/28);Darier病型棘层松解占10.7%(3/28).28例患者合并乳腺癌者21例(75.0%).明确治疗且随访时间超过1年的20例患者中18例无复发.结论 AMPD可能更易累及左侧乳房,最常合并的病理类型为经典模式型.AMPD中Hailey-Hailey病型棘层松解最为常见.AMPD易伴发乳腺癌,但治疗后复发率低.

abstractsObjective To investigate the clinicopathological features of acantholytic mammary Paget′s disease (AMPD).Methods From January, 2010 to October, 2016, a total of 28 patients were diagnosed as AMPD in the Department of Dermatology, Peking Union Medical College Hospital. The clinical and histopathological data of these patients were analyzed retrospectively.Results The patients were all female. The mean age of onset was (51±15)years (range, 24 to 78 years). The median course of disease was 10.5 months (range, 3 months to 2 years). All the cases were unilaterally involved. The ratio of left breast involvement to the right breast involvement was 1.55∶1. In histopathological examination, all the cases showed acantholytic pattern. In addition to that, the prototypical pattern accounted for 50.0% (14/28) of all the AMPD cases. The frequencies of other different accompanied histopathological patterns were: 3 (10.7%) upper nest, 2 (7.1%) budding, 1 (3.6%) tall nest, and 1 (3.6%) sheet-like. Hailey-Hailey-disease-like acantholysis was observed in 18 patients (64.3%), whereas pemphigus-vulgaris-like acantholysis was noted in 7 patients (25.0%) and Darier′s-disease-like acantholysis in 3 patients (10.7%).About 75.0% (21/28) of the AMPD cases were found to be accompanied with underlying breast cancers. There was no recurrence in 18 of 20 patients who completed treatment and were followed up for over 1 year.Conclusions AMPD might involve the left breast more frequently. It is mostly associated with the prototypical pattern of mammary Paget′s disease. Hailey-Hailey-disease-like acantholysis may be the most frequent subtype of AMPD. Most AMPD cases are associated with underlying breast cancers, but with a low recurrence rate after treatment.