title: [Clinicopathological features of acantholytic mammary Paget's disease: a report of 28 cases].
abstract: Objective: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.
authors.affiliation: Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
authors.forename: Y P
authors.initials: YP
authors.lastname: Zeng
authors.forename: C
authors.initials: C
authors.lastname: Chen
authors.forename: K
authors.initials: K
authors.lastname: Fang
authors.forename: Q N
authors.initials: QN
authors.lastname: Jia
authors.forename: D L
authors.initials: DL
authors.lastname: Ma
mesh_terms: D000051:Acantholysis / Q000473:pathology*; D000328:Adult; D000368:Aged; D001943:Breast Neoplasms / Q000175:diagnosis / Q000473:pathology*; D007644:Darier Disease; D005260:Female; D006801:Humans; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D010144:Paget's Disease, Mammary / Q000175:diagnosis / Q000473:pathology*; D010392:Pemphigus; D055815:Young Adult
keywords: Acantholysis; Clinicopathological features; Paget′s disease, mammary