This disease appears as a red, velvety area with white islands of tissue on the vulva. At times it may be pink, and occasionally there are moist, oozing ulcerations that bleed easily. Most people with the condition will experience itching and soreness. Almost all patients are postmenopausal, Caucasian women. The cause of this disorder is unknown, and it is usually diagnosed by a biopsy. The typical treatment is surgery.

Radiation therapy and chemotherapy are not considered to be radical, whereas surgical resection of the tumor with abdominal lymphadenectomy is highly invasive 2 , 3. Paget's disease of the vulva is classified into primary and secondary, which may be distinguished using immunohistochemistry 3 — 5. Primary Paget's disease is subdivided into three categories, namely intraepithelial Paget's disease IEP , IP, and Paget's disease as a manifestation of an underlying adenocarcinoma of a skin appendage or a vulvar gland 4 — 6. While IEP is usually treated with surgery and its prognosis is relatively good, IP is associated with a poor prognosis 1 , 3 and a treatment strategy for advanced IP has not yet been standardized 2 , 6. Thus, more effective and less invasive treatments for IP are required.

Try out PMC Labs and tell us what you think. Learn More. Vulvar Paget's disease is very rare malignancy originating in vulvar apocrine-gland-bearing skin cells or as a manifestation of adjacent primary anal, rectal or bladder adenocarcinoma. Vulvar Paget's disease is most commonly seen in postmenopausal Caucasian females and appears clinically as red, eczematous and pruriginous — and sometimes painful — lesions.

Jump to navigation. It is most common in postmenopausal white women. It is an intraepithelial layer of cells that forms the surface or lining of an organ condition that can present as white and red scaly areas on the vulva that may be itchy and painful.