Introduction

Vulval extramammary Paget’s disease (EMPD) is a nonsquamous intraepithelial lesion of the vulva. It is an uncommon premalignant skin disorder with invasive potential. All areas that contain apocrine glands can be affected, such as the vulva, perianal region, penis, scrotum, perineum and axilla, but it most commonly occurs on the vulva. Extramammary Paget’s disease of the vulva is defined by the presence of so‐called Paget cells in the epithelium. Vulval EMPD spreads in an occult manner, often with more unseen lesions extending beyond the apparent edges. Vulval EMPD can be subclassified into primary or secondary disease. Primary disease originates on the vulva and secondary EMPD is due to a noncutaneous neoplasm, often of adjacent sites, such as the bladder or rectum. Interventions can be surgical, or noninvasive techniques may be applied. The purpose of the treatment is to remove the disease but also to reduce side effects from radical surgery. There is hardly any consensus on treatment.

Epidemiology

Even in a vulval clinic, EMPD is seen in less than 1% of the cases. It affects mainly white postmenopausal women, with a median age of 72 years. The true incidence and prevalence remains unknown. To date, only a couple of hundred cases are recorded in the literature. There is an uncertain association between vulval EMPD and distant tumours, such as those of the breast, pancreas, endometrium, bladder, stomach and rectum. The association is variable across the literature, ranging from 0–50%.

Histological features