James Paget was the first to describe Paget disease (PD) in 1874, while extramammary Paget disease (EMPD) was first described by Crocker in 1888 [1]. The condition consists of an intraepithelial adenocarcinoma. In general, EMPD lesions are found in areas such as the vulva, anus, perianal region, and axillae in which the density of apocrine glands is high. In women, the most common site of EMPD is the vulva; however, EMPD is responsible for less than 1% of all vulvar neoplasms [2]. Diagnosis of EMPD usually occurs between 50 and 80 years of age, with the disease being more common in Caucasian women [3]. The most common clinical symptom is pruritus. The lesion may be erythematous or eczematous, with islands of hyperkeratosis [4]. Surgical resection with wide margins is considered the standard treatment; however, successful surgical excision of the disease is a challenge and recurrences are com- mon. Alternative treatments such as photodynamic therapy, laser therapy, radiotherapy, topical treatments such as 5% imiquimod cream, or even chemotherapy have been the subjects of debate and it is important to evaluate the available evidence [5].

Figure 1: Extra Mammary Pagets Disease.