A 60-year-old woman presented with vulvar pruritus for 1 year that is now constant. On physical examination, there is one large red lesion surrounded by white epithelium (Figure 88-1). A 3-mm punch biopsy was done of the white island within the red lesion using local anesthesia. The pathology showed Paget disease of the vulva. The patient underwent a wide local excision of the involved area and no malignancy was found.
Paget disease of the external genitalia is an uncommon primary cutaneous adenocarcinoma of apocrine gland-bearing skin. The most commonly involved site is the vulva, although perineal, perianal, scrotal, and penile skin may also be affected. Less commonly, the axilla, buttocks, thighs, eyelids, and external auditory canal may be found.1 It is morphologically and histologically identical to Paget disease of the nipple except for the anatomic location.
Extramammary Paget disease.