Extramammary Paget Disease: Causes, Symptoms, and Treatment
Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Extramammary Paget Disease?
A dermatologist, a skin doctor, usually diagnoses and treats extramammary Paget disease. A surgeon or an oncologist, a cancer doctor, may also help with treatment. A gynecologist, a doctor for the female reproductive system, may help when the vulva is affected. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is extramammary Paget disease?
Extramammary Paget disease (EMPD) is a rare cancer of the skin. It usually starts in skin areas that have apocrine glands, a type of sweat gland. These areas include the vulva, scrotum, and penis. The area around the anus, the armpit, and the navel can also be involved.
Under the microscope, EMPD looks very similar to Paget disease of the breast. The main difference is where the rash is. Paget disease of the breast happens around the nipple.
In the early stages, EMPD usually grows slowly. Most cases are found as carcinoma in situ, meaning the cancer cells have not grown deeper into the skin.
Symptoms
EMPD often shows up as a long-lasting, eczema-like rash. Signs can include:
- Red, well-defined patches of skin.
- Crust, scale, or raw areas on the red skin.
- Lighter or darker patches of skin in some cases.
- Itching or tenderness.
- Lumps or deep sores in the later stages.
The rash can look like eczema, psoriasis, or a fungal infection. Around 10% of people with EMPD have no symptoms.
Causes and who gets it
EMPD can be primary or secondary. Primary EMPD starts in the top layer of the skin. Secondary EMPD comes from cancer cells that spread to the skin, or grow into it, from a cancer inside the body. Examples are cancers of the colon or the urinary tract.
About 25 percent of EMPD is linked with an underlying cancer.
EMPD usually affects older adults. The average age at diagnosis is 60 to 70 years. In studies of Caucasian populations, EMPD is more common in women. Studies in Asia report more men.
How doctors diagnose it
EMPD can look like other skin problems, so a careful exam is needed. Doctors check every area where it can develop, because EMPD can appear in more than one place.
The diagnosis depends on a close look at skin tissue under a microscope. Doctors look for Paget cells, which are large unusual cells in the top layer of skin. Special lab stains help tell EMPD from melanoma and other skin conditions.
Doctors sometimes take extra skin samples around the rash, called mapping biopsies, because the edges can be hard to see.
EMPD is linked with other cancers. Doctors may think about checking for cancer inside the body, such as in the colon, rectum, prostate, or bladder.
Treatment
Surgery is the main treatment for the skin lesion. Wide local excision removes the lesion and a margin of normal skin around it. It has long been the standard treatment.
Mohs micrographic surgery is another option. The surgeon checks the whole edge of the tumor under a microscope during the operation.
Some people cannot have surgery, or choose not to. Other options include radiation therapy, imiquimod cream, and photodynamic therapy. Photodynamic therapy uses a light-sensitive medicine and light to destroy tumor cells. It may help with symptoms but may not cure EMPD. Its side effects include pain and sensitivity to light, which could be severe.
Using creams such as imiquimod before surgery has been linked with a higher chance of the cancer coming back.
Removing the lymph nodes is a standard treatment if the cancer spreads to nearby lymph nodes. Chemotherapy has been used when the cancer has spread to distant parts of the body, but it does not work well enough.
There is no clear research evidence showing which treatment is best. Long-term follow-up and careful exams are needed.
When to see a doctor
A long-lasting, eczema-like rash around the genitals or another place where EMPD can develop needs a doctor's exam. EMPD can look like eczema, psoriasis, or a fungal infection.
Lumps or deep sores can happen in the later stages, so have them checked right away. If you have been diagnosed with EMPD, keep your follow-up visits.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. Such a doctor can look at a rash that has not gone away, help set up a visit with a skin doctor, and help coordinate your care among specialists over time.
Common questions
Is extramammary Paget disease cancer?
Yes, it is a rare type of skin cancer. Most cases are found early, in the top layer of skin, where it usually grows slowly.
Is it the same as Paget disease of the breast?
No, they look very similar under the microscope, but the rash is in a different place. Paget disease of the breast is around the nipple. Extramammary Paget disease can happen in the vulva, the area around the anus, the scrotum, the penis, and the armpit.
Can extramammary Paget disease spread?
Yes, it can. Once Paget cells grow deep into the skin, spread to nearby lymph nodes and distant parts of the body often develops. People with distant spread have a poor outlook because chemotherapy has limited effect.
Can extramammary Paget disease be mistaken for eczema?
Yes, it often looks like eczema. It can also look like psoriasis or a fungal infection. Doctors need to study skin tissue under a microscope to make the diagnosis.
Sources
- NIH Genetic and Rare Diseases Information Center - Extramammary Paget disease
- National Library of Medicine (PubMed Central) - Extramammary Paget’s Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
