Primary extramammary Paget disease (EMPD) is a form of intraepithelial adenocarcinoma. Different morphological changes may accompany EMPD, including the presence of syringoma-like structures. The authors report 10 cases of EMPD, all of which manifested syringoma-like structures within the dermis both in areas involved by the carcinoma and beyond, including at the margins of the excisions. All patients were women, whose ages ranged from 49 to 93 years (median 75 years). The possible pathogenesis of the syringoma-like lesions is discussed. Awareness of these structures in vulvectomy specimens for EMPD is important to prevent misinterpretation of the syringoma-like lesions as an invasive component of the EMPD.

*Department of Pathology Clinical Research and Practical Center for Specialized Oncological Care Saint Petersburg Russia; †Department of Pathology Medical Faculty Saint Petersburg State University Saint Petersburg Russia; ‡Department of Pathology BC Cancer Agency Vancouver British Columbia Canada; §Department of Histopathology King Edward Memorial Hospital Perth Australia; ¶Department of Pathology Division of Dermatopathology Medical College of Wisconsin Milwaukee WI; ‖Sikl's Department of Pathology Bioptical Laboratory Pilsen Czech Republic; **Sikl's Department of Pathology Medical Faculty in Pilsen Charles University Prague Pilsen Czech Republic; and ††Department of Dermatology University Hospital Zurich Zurich Switzerland

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