Paget’s disease of the vulva

Paget’s disease of the vulva is a skin cancer that develops from glandular cells, most often occurring in Caucasian (white) women post menopause. The disease looks like a genital rash, like red velvet with small white or pink ‘islands’ on vulvar tissue. Sometimes these islands may ooze, appear like ulcers, or bleed easily. It is often itchy and sore.

This condition may also be called extramammary Paget’s disease of the vulva, since Paget’s disease was originally discovered around the breast, looking like a rash around the nipple – a sign of breast cancer.

      Symptoms of Paget’s disease of the vulva

  • There may be no symptoms
  • Itching
  • Cracked skin can be irritated, sore or cause pain
  • Bleeding, especially from cracked skin

     How Paget’s disease looks

  • The rash can be red, scaly, or crusty
  • Soggy skin, with white areas
  • Rash usually on outer labia (vaginal lips) on one or both sides
  • Rash can be found on the anus and the inner surface of the vulva
  • Not deep into the skin layers (but then it is called invasive extramammary Paget’s disease)
  • Invasive extramammary Paget’s disease looks like a skin thickening or lump around the rash area
  • It may be mistaken for eczema or psoriasis
  • Red velvet areas with white islands
  • Can be pinkish like eczema

The redness is caused by blood being brought closer to the surface of the mucous membrane on the inside of the vulva. The area has what are called hyperkeratotic plaques, which means extra keratin is deposited in the skin, much like a callous – hyperkeratotic skin is usually a normal thickening response of the skin, usually to help prevent further injury.

     Diagnosis of Paget’s disease
These unusual tumours have unknown origins, with diagnosis most often via biopsy – a piece of rashy skin is removed and examined under a microscope, using a small injection of local anaesthetic. More than one biopsy may be required. The lab will review the sample, with the lab technician looking for the specific pattern this disease causes that differentiates it from eczema.

Because this condition is associated with other forms of cancer, you will be checked all over – breasts, anus, rectum, vagina, etc. An ultrasound or CT scan may be performed to check your abdomen and pelvis, and the bowel will be checked using a colonoscopy. A mammogram will examine your breasts. Any other tests will be discussed with your doctor.

     Treatment of Paget’s disease
Treatment is usually surgery, with skin grafts possibly necessary to close the wound. The edges of the growth can be really hard to see, so a special surgical procedure, Mohs microscopic surgery, may be used to help find the edges of the tumour. Surgery will be required for invasive extramammary Paget’s disease of the vulva, and wave-based therapies may be suggested.

There are creams to treat this disease, with one in particular being Imiquimod cream that supports the immune system to kill the abnormal cells. It is not licensed to treat Paget’s disease of the vulva yet, but can be utilised as an adjunct and is available. CO2 lasers, photodynamic therapy, radiotherapy and chemotherapy are all options, along with the creams.

It is very important to get all the follow-up care your doctor requires of you – let loose, this problem will not go away by itself, and you cannot treat it by yourself.

. Recurrence rates are high. Vulvar Paget’s disease makes up between one and eight per cent of all malignant vulvar tumours, and is linked with another underlying cancer in 20-30 per cent of cases.

     The facts on Paget’s disease

  • Extramammary Paget’s disease of the genitals is a rare form of cancer, usually originating in the epidermis and glands
  • It is associated with other forms of cancer
  • Between 1962 and 1982, there were 196 cases reported in English medical literature
  • This disease usually occurs in elderly people, and can occur in men and women predominantly on the vulva and perineum
  • About 26 per cent of people with this form of cancer will die, leaving three-quarters surviving
  • Twenty-four per cent of those with this disease have an associated underlying malignant tumour formed from glandular structures in epithelial (skin) tissue
  • Those people with an associated malignant tumour in a glandular structure have an increased mortality rate – the risk of death increases to 46 per cent, leaving – still – 55 per cent (over half) surviving
  • Another underlying internal malignancy is found in 12 per cent of those with Paget’s disease of the vulva
  • Invasive Paget’s disease exists in 15 per cent of women with Paget’s disease of the vulva
  • The location of the underlying internal malignancy seems to be closely linked to the location of the Paget’s disease, for example if the lesions appear near the perineum and anus, it is associated with an underlying cancer of the digestive system; if it appears on the penis or scrotum, a genitourinary cancer
  • It is sometimes associated with cancer of the sweat glands, and less commonly, the urethra, bladder, rectum or vagina