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.
Surgical excision is recommended. Many studies have been demonstrated a high rate of positive margins despite radical surgery. Imiquimod is also efficacious, especially in women with recurrent disease after surgery or in those unable to undergo a surgery.
- 1.Paget J. On disease of the mammary areola preceding cancer of the mammary gland. Bartholomew’s Hosp Rep. 1874;10:87–9.Google Scholar
- 3.Crocker HR. Paget’s disease, affecting the scrotum and penis. Trans Pathol Soc Lond. 1889;40:187–91.Google Scholar
- 15.Edey KA, Allan E, Murdoch JB, Cooper S, Bryant A. Interventions for the treatment of Paget’s disease of the vulva. Cochrane Database Syst Rev. 2013;10:CD009245.Google Scholar