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Clinical Practice Points:

* Extramammary Paget disease is a rare condition most often found in the penile and vulvar regions but is not necessarily limited to these locations.

* The nonspecific symptoms and visual presentation can mimic a variety of other dermatological conditions, often delaying diagnosis.

* Immunohistochemical staining for markers including cytokeratin 7 are becoming increasingly important in making the diagnosis.

* The current mainstay of therapy is surgical excision with wide margins.

* Multiple new modalities are being tested to reduce the need for operative definitive therapy, potentially reducing morbidity.

(C) 2015Elsevier, Inc.