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Rev Bras Ginecol Obstet 2016; 38(10): 524-528
DOI: 10.1055/s-0036-1593884
Case Report
Thieme Publicações Ltda Rio de Janeiro, Brazil

Extramammary Paget Disease of the Vulva - Case Report

Relato de Caso: Doença de Paget Extramamária da Vulva
Bianca Ruschel Hillmann
1   Department of Gynecology and Obstetrics, Residency Program of the Hospital Universitário Professor Polydoro Ernani de São Thiago, Florianópolis, SC, Brazil
,
Amanda Amaro Pereira
2   Department of Pathology, Residency Program of the Hospital Universitário Professor Polydoro Ernani de São Thiago, Florianópolis, SC, Brazil
,
Luiz Fernando Sommacal
3   Department of Gynecology and Obstetrics, Health Sciences Center, Universidade Federal de Santa Catarina, Florianópolis, SC, Brazil
› Institutsangaben
Weitere Informationen

Publikationsverlauf

22. März 2016

22. September 2016

Publikationsdatum:
10. November 2016 (online)

   Lizenzen und Reprints

Abstract

Extramammary Paget disease (EPD) is a rare malign neoplasm that may affect the vulva and has manifestations common to benign diseases such as itching, pain and eczema. This leads to delay in diagnosis and consequent worse prognosis. The definitive diagnosis is obtained by biopsy of the vulva, which shows Paget cells. The treatment of choice is wide excision with margins, which leads to sequelae, functional and aesthetic. Recurrence is common. This article reports the case of a 48-year-old female patient with history of vulvar itching for the past 2 years, who had been submitted to various treatments for benign pathologies. The patient was biopsied and was diagnosed with extensive EPD, being submitted to vulvectomy. This article aims to draw attention to the need for biopsy of pruritic vulvar lesions that do not respond to usual treatment.

Resumo

A doença de Paget extramamária (DPEM) é uma neoplasia maligna incomum que pode acometer a vulva, e que tem manifestações comuns a doenças benignas como prurido, dor e eczema. Isto leva a atraso no diagnóstico e, consequente, piora do prognóstico. O diagnóstico de certeza se dá pela biópsia de vulva que evidencia células de Paget. O tratamento de escolha é a excisão com margens amplas, que leva a sequela funcional e estética. A recidiva é frequente. Este artigo relata o caso de uma paciente do sexo feminino, de 48 anos, com história de prurido vulvar há 2 anos com diversos tratamentos para patologias benignas. A biopsia da paciente confirmou diagnóstico de DPEM extensa sendo submetida a vulvectomia. Este artigo visa chamar a atenção para a necessidade de biópsia das lesões vulvares pruriginosas que não respondem ao tratamento habitual.

Keywords

extramammary Paget disease - vulvar neoplasms - pruritus vulvae - vulvar Paget disease

Palavras-chave

doença de Paget extramamária - neoplasias vulvares - prurido vulvar - doença de Paget vulvar
 

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