Have you noticed a red, scaly, or crusty patch of skin on your lower belly, underarms, or around your private areas? Does the spot slowly grow larger over time, sometimes accompanied by a persistent itch? If this sounds familiar, you may want to learn about a rare skin condition called Extramammary Paget's Disease, or EMPD for short. While it is uncommon, understanding the signs can help you catch it early and get the right care.
Finding a strange skin spot in a sensitive area can feel scary, embarrassing, or confusing. You are definitely not alone in feeling this way. Because EMPD can look and feel a lot like common eczema or a simple skin rash, it often takes time to diagnose properly. Let us walk through everything you need to know about EMPD in clear, simple terms.
Key Takeaways About EMPD.
- EMPD is a rare skin condition that usually causes a slow-growing, red, scaly, or crusty skin patch.
- It most often appears in areas with many sweat glands, such as the groin, vulva, or anal region.
- A skin biopsy is required to confirm the diagnosis by looking for abnormal Paget cells under a microscope.
- Surgery is the primary treatment method, and ongoing follow-up visits are crucial because EMPD can return.
- Catching the condition early and working closely with your doctor helps ensure the best possible long-term health outcome.
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What is Extramammary Paget's Disease (EMPD)?
Simply put, EMPD is a rare skin condition that sometimes connects to underlying cancer, though not always. It causes a red, scaly, or crusty patch on your skin that slowly enlarges over time. Sometimes, it can take years after symptoms first appear before doctors make a clear diagnosis.
The name "Paget's Disease" honors Sir James Paget, a brilliant 19th-century British doctor. He made major contributions to pathology, and several medical conditions bear his name. The word "extramammary" simply means "outside of the breast." Doctors use this distinction to avoid confusing it with Paget's disease of the breast.
Studies show that between 7 percent and 40 percent of people diagnosed with EMPD have an underlying internal cancer. Even so, EMPD does not automatically mean you have another serious illness. Getting prompt medical care helps relieve your symptoms and lets your doctor check for any underlying health issues.
What Are the Main Types of EMPD?
Doctors divide Extramammary Paget's Disease into two main categories based on how it develops:
1. Primary EMPD: This type starts right in your skin cells on its own without any outside cause.
2. Secondary EMPD: This type develops because of another cancer growing somewhere else inside your body.
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Check Availability →Who is Most Likely to Get EMPD?
While anyone can develop EMPD, doctors diagnose it most often in specific groups of people:
- Adults between 50 and 80 years old.
- Women more often than men.
- Asian men in certain populations.
- White people.
Even among these groups, EMPD remains a very rare medical condition.
Why Does EMPD Happen?
What Causes Primary EMPD?
Medical experts still do not know the exact cause of primary EMPD. However, they suspect it begins within specific specialized skin cells, including:
- Apocrine gland duct cells: These cells live inside sweat glands near hair follicles.
- Keratinocyte stem cells: These cells help repair and regenerate your skin.
- Toker cells: These cells reside in the tissue around the nipple and vulva.
What Causes Secondary EMPD?
Secondary EMPD occurs because of an internal cancer elsewhere in your body. For example, about one-third of people with secondary EMPD trace their condition back to rectal cancer. Other related internal cancers include:
- Anal cancer.
- Bladder cancer.
- Cervical cancer.
- Ovarian cancer.
- Prostate cancer.
- Uterine cancer.
What Are the Symptoms of EMPD?
The most common sign of EMPD is a skin patch that looks very much like eczema. Research shows that roughly 65 percent of patients develop EMPD on the vulva. This about 20 percent develop it around the anus.
Your affected skin may show several distinct visual characteristics:
- Crusty texture.
- Red coloration.
- Scaly flakes.
This patch expands very slowly, and its outer edges can look irregular. As the condition advances, you might notice deeper open sores or small lumps. Additional physical symptoms often include:
- A burning feeling in your skin.
- Irritation and redness.
- Persistent itching.
- Pain or tenderness when touched.
- Swelling in the area.
EMPD most often affects areas with high concentrations of apocrine sweat glands. While the vulva is the most common site, it can also appear on:
- The anus.
- The penis.
- The perineum.
- The scrotum.
In rare instances, EMPD appears on other parts of the body, such as:
- The abdomen.
- The armpit.
- The bellybutton.
- The buttocks.
- The ear, eyelid, face, or scalp.
- The lower chest.
- The knee or thigh.
Keep in mind that about 10 percent of people with Extramammary Paget's Disease experience zero symptoms at all.
How Do Doctors Diagnose EMPD?
Your doctor will start by asking about your medical history and performing a physical examination. If they suspect EMPD, they will perform a skin biopsy. During a biopsy, your doctor removes a tiny sample of your skin so a specialist can study it under a microscope.
Under the microscope, a pathologist looks for large, unusual cells known as Paget cells. They also use special chemical tests, called immunohistochemistry, to check how these cells react and confirm the exact diagnosis.
How Do Doctors Check for Underlying Cancer?
Once your doctor confirms an EMPD diagnosis, they will recommend several follow-up tests to check for internal cancer. These tests may include:
- Ultrasound imaging.
- A mammogram.
- A Pap smear.
- A colonoscopy or proctoscopy.
- A CT scan.
- A cystoscopy to view your bladder.
- A prostate exam.
- A retrograde pyelogram to view your kidneys and urinary tract.
- An ultrasound or needle biopsy of your lymph nodes.
- An upper endoscopy to check your throat, esophagus, and stomach.
What Are the Treatments for EMPD?
Doctors typically treat Extramammary Paget's Disease using surgery.
- Mohs surgery: Your surgeon removes the affected skin layer by layer, checking each layer under a microscope until no cancer cells remain. This method protects as much healthy skin as possible.
- Wide local excision: Your surgeon removes the EMPD patch along with a border of healthy tissue surrounding it, then closes the wound with stitches.
Sometimes, surgeons must remove a larger area of skin and tissue. For instance, women with vulvar EMPD might need a partial or total vulvectomy.
If other health conditions prevent you from having surgery, your doctor may suggest alternative treatments:
- Immunotherapy to boost your immune defenses.
- Laser skin resurfacing.
- Photodynamic therapy.
- Radiation therapy.
- Topical cancer medicines.
Most importantly, if you have an underlying internal cancer, you must receive separate, specialized treatment for that condition as well.
Can You Prevent EMPD?
Unfortunately, no known methods exist to lower your risk or prevent Extramammary Paget's Disease from developing.
What is the Long-Term Outlook for EMPD?
After receiving treatment for EMPD, you will need regular follow-up visits with your doctor. The exact schedule depends on where the disease appeared on your body. It is quite common for EMPD to return in or near the exact same spot. Therefore, sticking to your follow-up schedule is vital.
While EMPD spreading to other parts of your body is rare, it can sometimes travel to your lymph nodes or other organs. Your doctor can give you the most accurate outlook based on your specific health details.
| Aspect of Care. | What to Expect. |
|---|---|
| Diagnosis Method. | Skin biopsy reviewed by a pathologist using special stains. |
| Primary Treatment. | Mohs surgery or wide local excision to remove abnormal cells. |
| Recurrence Risk. | Moderate chance of returning, requiring lifelong skin checks. |
| Global Support. | Guidance from major health organizations like the NHS and Mayo Clinic. |
Can EMPD Return After Treatment?
Yes, EMPD can recur even years after successful treatment. In fact, it can return 15 years or more down the road. This is why doctors emphasize long-term monitoring and regular skin examinations.
How Can You Care for Yourself If You Have EMPD?
The best thing you can do is attend every scheduled follow-up appointment with your doctor. If your doctor spots early signs of EMPD returning, they can treat it quickly.
When Should You See a Doctor?
If you notice any new skin spots or changes in your existing symptoms, schedule an appointment with your doctor right away.
Emergency Red Flags.
If you experience severe pain, heavy bleeding from a skin lesion, sudden rapid swelling, or signs of a severe systemic infection, call 911 (US/Canada) or 999 (UK), or go to your nearest Emergency Department right away.
Sources & References:
- Cleveland Clinic. (2023). Extramammary Paget's Disease (EMPD): Symptoms & Treatment.
- Mayo Clinic. (2023). Paget's Disease of the Skin: Diagnosis and Management.
- National Health Service (NHS UK). (2023). Skin conditions and specialist dermatology care.
- Centers for Disease Control and Prevention (CDC). (2024). General health and preventive screenings.
- National Institutes of Health (NIH) / PubMed Central. (2022). Clinical review on the management of Extramammary Paget's Disease.
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