Extramammary Paget’s disease of the vulva: Persistent vulvar itching requires a skin biopsy!

Abstract

Our case highlights the need to perform a skin biopsy in patients presenting with persistent vulvar itching, especially if they have not responded to conventional treatment for benign conditions.

Our case highlights the need to perform a skin biopsy in patients presenting with persistent vulvar itching, especially if they have not responded to conventional treatment for benign conditions.

1. CASE REPORT

We describe the case of a woman with a chronic eczematous plaque on the vulva. Histopathology revealed a diagnosis of extramammary Paget's disease, a rare adenocarcinoma that may be a manifestation of a distant cancer. Skin biopsy must be performed in patients with persistent vulvar itching.

A 50‐year‐old woman presented with a 13‐year history of an itchy genital lesion, unresponsive to topical corticosteroids and fluconazole. On examination, there is an erythematous, eczematous plaque on the left labia of the vulva (Figure 1). Biopsy showed epidermal infiltration by large atypical cells with clear cytoplasm and prominent nuclei (Figure 2 arrows). Immunohistochemistry was compatible with extramammary Paget's disease (EMPD) (Figure 3). Colonoscopy, abdominal‐pelvic CT scan, mammography, and cystoscopy were negative. The patient underwent wide local excision with 2‐cm safety margins that were positive on histopathology. Radiotherapy was delivered to the perineum at 50 grays (Gy) in 30 fractions over 5 weeks. Two years later, she had a biopsy‐confirmed recurrence treated with a new cycle of radiotherapy.

Figure 1.

Erythematous, eczematous plaque on the left labia of the vulva

Figure 2.

Epidermal infiltration by large atypical cells with clear cytoplasm and prominent nuclei (Paget cells; HE 400×)

Figure 3.

Immunohistochemistry positive for cytokeratin‐7 (CK‐7) and carcinoembryonic antigen (CEA), and negative for HMB‐45, compatible with extramammary Paget's disease

EMPD is a rare adenocarcinoma that accounts for <1% of vulvar malignancies. ¹ It can be of primary cutaneous origin or, less frequently, a manifestation of an underlying or distant cancer. The disease may go undiagnosed for years as it can mimic many benign conditions, so histopathological confirmation is required. ² Surgery remains the best treatment option; however, recurrence is frequent. Our case highlights the need to perform a skin biopsy in patients presenting with persistent vulvar itching.

CONFLICT OF INTEREST

None declared.

AUTHOR CONTRIBUTIONS

Both authors made substantial contributions to the preparation of the manuscript. CVK: was the patient's primary physician and revised and approved the final version of the manuscript. CH: performed the literature search and drafted the manuscript.

Vera‐Kellet C, Hasbún C. Extramammary Paget’s disease of the vulva: Persistent vulvar itching requires a skin biopsy!. Clin Case Rep. 2020;8:1325–1326. 10.1002/ccr3.2862