Paget's disease of the vulva

Abstract

Paget’s disease of the vulva is a rare vulvar neoplasm most commonly seen in postmenopausal women. The mean age at diagnosis has been reported to range from 50 to 80 years. Women commonly present with a well-demarcated pink eczematous lesion with white islands of hyperkeratosis, often accompanied by pruritus, vulvar pain, and erythema. Some women remain asymptomatic. Page’s disease may present as type 1, a primary cutaneous form, with two subtypes: 1a, intraepithelial Paget’s disease; 1b, intraepithelial disease with invasion; and 1c, intraepithelial Paget’s disease with underlying adenocarcinoma of a skin appendage or subcutaneous gland. Type 2 is metastasis from underlying malignancy of the gastrointestinal tract, and 2b is metastasis from the urogenital tract or adenocarcinoma originating elsewhere. Regardless of treatment, 12–62% of patients develop recurrent disease.