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.

Fig. 1

Clinical presentation: erythematous plaque covered by fine and whitish scales, with some areas of erosion.

Fig. 2

Histopathological and immunohistochemistry features: (a) Pagetoid infiltration of the epidermis by pleomorphic large cells, isolated or in nests, with clear cytoplasm and a prominent nucleolus (hematoxylin and eosin x100); (b) Pagetoid cells (hematoxylin and eosin x400); (c) Positive cytokeratin 7 staining (CK7, x100).

Fig. 3

Clinical presentation: Paget disease of the vulva relapse (erythematous plaque) after total vulvectomy and radiotherapy.