棘层松解性乳房Paget病28例临床病理特点分析  

Clinicopathological features of acantholytic mammary Paget＇s disease： a report of 28 cases

作　　者：曾跃平[1] 陈程[1] 方凯[1] 贾倩楠[1] 马东来[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院皮肤科,100730

出　　处：《中华医学杂志》2017年第14期1076-1078,共3页National Medical Journal of China

基　　金：国家自然科学基金（81371723）;北京协和医院中青年科研基金（pumeh-2016-2.12）

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。Objective To investigate the clinieopathologieal features of acantholytie 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 histopathologieal data of these patients were analyzed retrospectively. Results The patients were all iemale. 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 eases showed acantholytie pattern. In addition to that, the prototypical pattern accounted for 50. 0% （ 14/ 28） of all the AMPD eases. 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. Halley- Halley-disease-like aeantholysis was observed in 18 patients （64. 3% ） , whereas pemphigus-vulgaris-like aeantholysis was noted in 7 patients （25.0%） and Darier＇ s-disease-like aeantholysis 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 treatnlent 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. Halley-Halley-disease-like aeantholysis 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.

关 键 词：佩吉特病 乳腺 皮肤棘层松解 临床病理特征 

分 类 号：R739.5[医药卫生—肿瘤]