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Vulvar Disease pp 319-322 | Cite as

Paget’s Disease of the Vulva

  • Mila Pontremoli Salcedo
  • Kathleen M. SchmelerEmail author
Chapter

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.

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.

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  • Mila Pontremoli Salcedo
    • 1
  • Kathleen M. Schmeler
    • 2
    Email author
  1. 1.Federal University of Health Sciences Porto Alegre (UFCSPA)/Irmandade Santa Casa de Misericordia de Porto AlegrePorto AlegreBrazil
  2. 2.Department of Gynecologic Oncology and Reproductive MedicineThe University of Texas MD Anderson Cancer CenterHoustonUSA

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