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Therapeutic Approach

Mammary Paget’s disease (MPD) and extramammary Paget’s disease (EMPD) are rare intraepithelial carcinomas.1-2 MPD is typically associated with underlying breast cancer and most commonly affects postmenopausal women.1 It is characterized by erythematous, eczematous, weeping or crusted plaques on the nipple and periareolar skin.1 In contrast, EMPD typically occurs in sites with a high concentration of apocrine glands, including the genital skin, axillae, and anus, with the vulva being the most frequently affected region.2 EMPD is seen most commonly in older Caucasian females and Asian males and typically occurs in the 6th to 8th decades of life.2 It can arise as a primary cutaneous adenocarcinoma or may be secondary to an underlying adenocarcinoma.2 Both MPD and EMPD can mimic inflammatory skin disorders and therapy is often delayed due a prolonged time to definitive diagnosis.1,2 The treatment of EMPD is based on the degree of extension and underlying malignancy. Treatments for localized disease include surgical resection, including Mohs micrographic surgery (first-line) and wide local excision (second-line), topical chemotherapy (reserved for poor surgical candidates), photodynamic therapy, and radiotherapy; however, recurrence rates can be high. Invasive disease, lymphovascular invasion, lymph node metastasis, and positive resection margins portend a worse prognosis.2 Management of MPD varies based upon the clinical and histologic features of the underlying breast cancer and will not be addressed in this chapter. See Table 106-1.

Table 106-1EMPD Treatment Table

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