A case of chronic and relapsing paget disease of the vulva

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.

Recuperação completa da doença de Paget extramamária decorrente da vulva (DPEM r-V) após tratamento com Imiquimode / Full recovery of recurrent extramammary Paget's disease of the vulva(rEMPD-V) after imiquimod treatment

Apesar de rara, a doença de Paget extramamária recorrente da vulva (DPEMr-V) é uma condição grave porque, subjacente à malignidade interna, podem acompanhar lesões cutâneas superficiais. A doença de Paget extramamária é uma condição caracterizada por erupção cutânea crônica tipo eczema de pele ao redor da região anogenital em homens e mulheres. Sob o microscópio,é muito parecida com o tipo mais comum da doença de Paget mamária, que ocorrena mama. A doença de Paget extramamária ocorre mais comumente em mulheres com idades entre 50 a 60 anos. Contudo, a excisão cirúrgica é o padrão geralmente aceito para a DPEMr-V. As taxas de recorrência da DPEMr-V são altas, apesar da intervenção cirúrgica agressiva. O tratamento tópico com imiquimod creme a 5% pode ser eficaz na remoção de lesões. Relatamos o caso de uma mulher de 72 anos com DPEMr-V comprovada por biópsia, tratada com sucesso com imiquimod, com aplicações três vezes por semana, durante 6 semanas.

Although rare, extramammary Paget's disease (EMPD) is a serious condition because underlying internal malignancy may accompany superficial cutaneous lesions.Extramammary Paget disease is characterised by a chronic eczema-like rash of the skin around the anogenital regions of males and females. Underthe microscope it looks very similar to the more common type of mammary Paget´s disease that occurs on the breast. Extramammary Paget disease mostcommonly occurs in women aged between 50-60 years. Although surgical excision is the generally accepted standard of care for EMPD. The EMPD-Vrecurrence rates are high despite aggressive surgical intervention, treatment with topical imiquimod 5 percent cream has reportedly been efficacious inclearing lesions.We report the case of a 72-year-old woman with biopsy-proven EMPD-V of the thigh treated successfully with imiquimod application thrice weekly for 6 weeks.