Vulvar Paget's Disease

Vulvar Paget's disease appears as a red (sometimes pink) velvety area with white islands of tissue on the vulva. Occasionally, there are moist oozing ulcerations that bleed easily. Almost all the patients affected are postmenopausal women; around half have itching symptoms.

Vulvar Paget's disease is an intra-epithelial neoplasm associated with atypical glandular cells of apocrine origin. The cause is unknown, but one-fifth of cases have associated underlying adenocarcinoma. There is also an association with extravulvar malignancies (e.g. of the rectum, urethra or bladder).

Diagnosis is by biopsy and the condition is best treated by wide local excision. However, since Paget's cells are frequently remote from the clinically evident disease, there is the potential for recurrence. Close follow-up after surgery is therefore required.

This particular photograph is from a 69-year-old Caucasian woman who presented with a localised area of vulvar pruritus. She enquired whether it was related to a condition that had been treated previously by simple vulvectomy; the diagnosis was subsequently confirmed as recurrent Paget's disease of the vulva.