Extramammary Paget Disease of the Vulva - Case Report

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.

The utility of high-frequency ultrasonography in preoperative assessment of vulvar Paget’s disease – a case report

“…Hopefully, in cases of PDV with more favorable location or smaller size, the HFUS could improve surgical outcome in terms of negative margins. Unfortunately, in most PDV cases reported in the literature, significant delay in the diagnosis leads to quite extensive vulvar lesions and more unfavorable outcome (14) .…”

Mohs micrographic surgery combined local flaps in treatment of EMPD: A retrospective case

“…EMPD was first described by James Paget, and usually occurs at apocrine glands‐rich areas, such as the penis, scrotum, vulva, perineum, and axillae of middle‐aged and older individuals (Hillmann, Pereira, & Sommacal, ). The typical clinical symptoms as reddish patches or gray‐white plaques, which are eczematoid, crusty scaling, or papillomatous (Kusatake et al, ; Laiyemo, ; Pena‐Romero, Dominguez‐Cherit, & Saeb‐Lima, ).…”

Vulvar Paget’s Disease Presenting with Fever and Left Inguinal and Peritoneal Lymphadenopathies

“…The time from onset to diagnosis of vulvar Paget’s disease is approximately 20 months [ 2 – 4 ] because it is extremely difficult to distinguish the skin lesions of Paget’s disease from benign conditions, such as contact dermatitis, fungal infections, or Bowen’s disease, using only gross physical findings without biopsy [ 1 , 5 ]. Therefore, biopsy is essential with recalcitrant vulvar skin lesions, despite usual treatments [ 6 ]. Furthermore, vulvar Paget’s disease could present only as inguinal lymphadenopathies as a chief complaint without vulvar symptoms [ 5 ], and patients’ embarrassment regarding examination of their pubic area could actually prevent physical examination [ 7 , 8 ].…”