CC BY 4.0 · Rev Bras Ginecol Obstet 2019; 41(06): 412-416
DOI: 10.1055/s-0039-1687861
Case Report
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

A Case of Chronic and Relapsing Paget Disease of the Vulva

1  Department of Dermatovenereology, Centro Hospitalar Universitário Lisboa Norte, Hospital de Santa Maria, Lisboa, Portugal
,
Maria Mendonça-Sanches
1  Department of Dermatovenereology, Centro Hospitalar Universitário Lisboa Norte, Hospital de Santa Maria, Lisboa, Portugal
,
Luís Soares-de-Almeida
1  Department of Dermatovenereology, Centro Hospitalar Universitário Lisboa Norte, Hospital de Santa Maria, Lisboa, Portugal
2  Institute of Molecular Medicine, Faculdade de Medicina de Lisboa, Lisboa, Portugal
,
Isabel Correia-Fonseca
1  Department of Dermatovenereology, Centro Hospitalar Universitário Lisboa Norte, Hospital de Santa Maria, Lisboa, Portugal
› Author Affiliations
Further Information

Publication History

05 December 2018

07 March 2019

Publication Date:
24 April 2019 (online)

  

Abstract

Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently affected location, it corresponds solely to 1 to 2% of all vulvar malignancies. A 72-year-old female patient was observed in our outpatient clinic with a 2-year history of an erythematous and pruritic plaque on the vulva. Histopathology and immunohistochemistry studies were compatible with extramammary Paget disease of the vulva. Associated neoplastic conditions were excluded. Due to multiple relapses, the patient was submitted to three surgical interventions, including a total vulvectomy, and to external radiotherapy. The present case illustrates the chronic and recurrent nature of extramammary Paget disease despite aggressive procedures as well as the challenge in obtaining tumor-free resection margins.