Understanding 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.

Also known as extramammary Paget’s disease, the condition appears as 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. Symptomatic skin is often itchy and sore.

Extramammary Paget’s disease is always symptomatic, with the most common symptom being pain.

Paget’s disease was originally discovered around the breast, looking like a rash around the nipple – a sign of breast cancer. Paget’s is found in older women.

Symptoms of Paget’s disease of the vulva

Pain
Itching
Cracked skin can be irritated, sore or cause pain
Bleeding, especially from cracked skin
Rash-like lesions

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 of vulvar skin is caused by blood being brought closer to the surface of the mucous membrane on the inside of the vulva.

The area has what is 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 the body’s attempt to try to prevent further injury.

Diagnosis of extramammary Paget’s disease

These unusual vulvar tumours have unknown origins, with diagnosis most often via a biopsy. A piece of rashy skin is removed and examined under a microscope using a small local anaesthetic injection. More than one biopsy may be required.

A lab reviews the sample, with the lab technician looking for the specific pattern this disease causes that differentiates it from eczema.

Because Paget’s disease of the vulva is associated with other forms of cancer, checking all over the body is important. Checks are made on the breasts, anus, rectum, vagina, and so on.

An ultrasound or CT scan may be performed to check the abdomen and pelvis, and the bowel will be checked using a colonoscopy. A mammogram will examine the breasts. Any other tests will be discussed with your doctor.

Treatment of Paget’s disease of the vulva

Treatment of Paget’s disease of the vulva is usually surgery, with skin grafts possibly necessary to close the wound. The edges of the growth (margins) can be hard to see, so a special surgical procedure, Mohs microscopic surgery, may be used to help find the edges of the tumour.

Surgery is almost always required for invasive extramammary Paget’s disease of the vulva. There are other management strategies, particularly for anyone for whom surgery is inadvisable.

There are creams to treat Paget’s disease, particularly Imiquimod cream, which supports the immune system in damaging abnormal cells.

Imiquimod is not licensed to treat Paget’s disease of the vulva yet, but can be utilised as an adjunct and is available. Vaginal application of Imiquimod can cause greater systemic symptoms and so tends to be best used on the vulvar and other external skin.

Wave-based therapies may be suggested. 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 – this problem will not go away by itself, and you cannot treat it alone.

Recurrence rates of Paget’s 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. It is not benign.

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.
Paget’s 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 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.

How can complementary and alternative medicine be used for Paget’s disease of the vulva?

Supportive natural therapies can be used alongside primary treatment by your primary practitioner. Make sure to report all herbs and supplements you are taking with all your practitioners to ensure safety. Complications can arise when incompatible treatments are being used concurrently.

Supportive treatments for cancer include antioxidants to combat the deleterious effects of chemotherapy or radiation therapy.

If you have had surgery, supporting wound healing and healthy tissue formation is important. Ensuring a nutritious, anti-inflammatory diet during treatments and healing and beyond is very important. Ensure plenty of critical nutrients for healing and repair.

Mental and emotional support during and after a cancer diagnosis is very important. Cancer of any kind is confronting, but cancer in such a private area can be even harder since it’s not as easy to talk about. There may be shame and embarrassment alongside grief and fear. Get support.

References^1–5

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Delport ES. Extramammary Paget’s disease of the vulva: An annotated review of the current literature. Aust J Dermatology. Published online June 4, 2012:9-21. doi:10.1111/j.1440-0960.2012.00898.x
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Chanda JJ. Extramammary Paget’s disease: Prognosis and relationship to internal malignancy. Journal of the American Academy of Dermatology. Published online December 1985:1009-1014. doi:10.1016/s0190-9622(85)70254-x
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Lopes Filho LL, Lopes IMRS, Lopes LRS, Enokihara MMSS, Michalany AO, Matsunaga N. Mammary and extramammary Paget’s disease. An Bras Dermatol. Published online April 2015:225-231. doi:10.1590/abd1806-4841.20153189
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Tebes S, Cardosi R, Hoffman M. Paget’s disease of the vulva. American Journal of Obstetrics and Gynecology. Published online August 2002:281-284. doi:10.1067/mob.2002.125700