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

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.