Extramammary Paget’s Disease

What is extramammary Paget’s disease?

Extramammary Paget’s disease (EMPD) is a rare type of skin condition that may be associated with different types of cancer. It usually causes a red, scaly or crusty area of your skin that grows slowly. Sometimes, EMPD isn’t diagnosed until many years after it starts to form.

EMPD is named for Sir James Paget, a famed 19th century British physician. Multiple forms of cancer carry Paget’s name because of his work in the field of pathology. “Extramammary” means “outside the breast,” and helps avoid confusion with other diseases named for Paget (such as Paget's disease of the breast).

About 7% to 40% of cases of extramammary Paget’s disease are associated with an underlying cancer. But EMPD isn’t always a sign of another disease. Getting treatment can help improve your symptoms and evaluate for underlying cancer.

What are the types of extramammary Paget’s disease?

There are two types of EMPD:

• Primary, which develops in your skin on its own.
• Secondary, which develops due to an underlying cancer.

Who might have extramammary Paget’s disease?

Everyone can develop extramammary Paget’s disease. However, EMPD is most likely to affect people who are:

• Between 50 and 80 years old.
• Designated female at birth (DFAB).
• Designated male at birth (DMAB) and are Asian.
• White.

How common is extramammary Paget’s disease?

EMPD is rare.

What causes primary extramammary Paget’s disease?

Providers don’t know the exact cause of primary extramammary Paget’s disease. They suspect it may form from specific types of cells found on certain areas of your body. Those cell types include:

• Apocrine gland duct cells: Sweat glands in your skin related to hair follicles.
• Keratinocyte stem cells: Cells that help with skin repair.
• Toker cells: Cells in the skin of your nipple and vulvar tissue.

What causes secondary extramammary Paget’s disease?

Secondary extramammary Paget’s disease results from an underlying cancer. One-third of EMPD cases result from rectal cancer. Other cancers that may cause secondary EMPD include:

• Anal cancer.
• Bladder cancer.
• Cervical cancer.
• Ovarian cancer.
• Prostate cancer.
• Uterine cancer.

What are the symptoms of extramammary Paget’s disease?

The main symptoms of EMPD are a skin rash very similar to eczema. Available research shows that about 65% of EMPD cases affect the vulva and 20% affect the area around the anus.

You may have areas of skin that are:

• Crusty.
• Red.
• Scaly.

The rash grows slowly and may have irregular borders. As EMPD progresses, deep sores called ulcers or nodules may occur. Other symptoms may include skin that’s:

• Burning.
• Irritated.
• Itchy.
• Painful or tender.
• Swollen (edema).

EMPD most often occurs where you have many sweat glands that open into your hair follicles (apocrine skin glands). It most commonly appears on your vulva. It can also be on the skin around your:

• Anus.
• Penis.
• Perineum.
• Scrotum.

In rare cases, EMPD may appear on your skin near other parts of your body, including your:

• Abdomen.
• Armpit.
• Bellybutton.
• Buttocks.
• Ear, eyelid, face or scalp.
• Lower chest.
• Knee or thigh.

About 10% of people with extramammary Paget’s disease don’t have any symptoms.

How is extramammary Paget’s disease diagnosed?

A healthcare provider will first ask about your symptoms and do a physical exam. If they suspect EMPD, they’ll do a skin biopsy. During a biopsy, they take a small sample of your skin to examine under a microscope.

Under the microscope, a pathologist will look for abnormal large cells with distinctive characteristics called Paget cells. They’ll also see how Paget cells react to various chemicals (immunohistochemistry) to correctly identify these cells.

How is cancer related to extramammary Paget’s disease diagnosed?

After your provider diagnoses you with EMPD, they’ll use other tests to see whether you may have an underlying cancer, including:

• Ultrasound.
• Breast screening (mammogram).
• Cervical screening (Pap smear).
• Colonoscopy or proctoscopy.
• CT scan.
• Cystoscopy.
• Prostate exam.
• Retrograde pyelogram.
• Ultrasound or needle biopsy of the lymph nodes.
• Upper endoscopy.

How is extramammary Paget’s disease treated?

Providers usually treat extramammary Paget’s disease with surgery, including:

• Mohs surgery: During this procedure, your surgeon removes the affected skin cells layer by layer. This technique helps save the surrounding healthy skin.
• Wide local excision: Your surgeon removes the area of EMPD along with some healthy tissue around it. They then sew together the edges of the wound.

Surgeons often need to remove a large area of skin and other tissue. Some people with EMPD of their vulva may need to have all or part of their vulva removed (vulvectomy).

If you can’t have surgery due to poor health or other conditions, your provider may suggest treatments such as:

• Immunotherapy.
• Laser skin resurfacing.
• Photodynamic therapy (PDT).
• Radiation therapy.
• Topical medications used to treat cancer (cytotoxic drugs).

Any underlying cancers will also need additional treatment.

How can I reduce my risk of extramammary Paget’s disease?

Unfortunately, you can’t reduce your risk of EMPD.

What can I expect if I have extramammary Paget’s disease?

After treatment of EMPD, you’ll need ongoing visits with your doctor depending on the location of EMPD. It’s not uncommon for EMPD to come back in the same area or surrounding area that was already treated, so follow-up is important.

It’s rare for extramammary Paget’s disease to spread (metastasize). But it may sometimes progress to lymph nodes or other organs. Other factors can also influence your outcome. Your healthcare provider is the best person to explain what’s most likely in your case.

What is the outlook if I have extramammary Paget’s disease?

It’s common for EMPD to reoccur years after you have initially. In some cases, it can return after more than 15 years, so your healthcare provider will likely recommend long-term monitoring and follow-up.

How do I take care of myself with extramammary Paget’s disease?

You can continue to take care of yourself by seeing your provider for regular follow-up appointments. If your provider sees any signs of reoccurrence of EMPD, prompt treatment can help.

When should I see my healthcare provider?

You should see your healthcare provider if you notice any new signs of EMPD or have any new symptoms.

A note from Cleveland Clinic

Having any kind of skin rash on sensitive or intimate parts of your body can be scary, embarrassing and frustrating. Extramammary Paget’s disease (EMPD) is often mistaken for other skin conditions and may not be properly diagnosed for quite some time. EMPD can be more serious if you also have an underlying cancer.

Fortunately, many people with EMPD live full lives after they receive treatment. Providers usually treat EMPD with surgery. If you do have an underlying cancer, you’ll also need cancer treatments. As it’s common for extramammary Paget’s disease to recur, seeing your provider for regular visits can help catch it early if it comes back.