Have you ever noticed a red, scaly, sometimes crusty patch on your skin, especially in the groin, armpits, or genital area? It seems to grow gradually, and sometimes itches, right? Okay, so today we're going to talk about a special skin condition like that. Doctors call it Extramammary Paget's Disease , or EMPD for short. It's a bit rare, but it's important to be aware of.
What is Extramammary Paget's Disease (EMPD)?
Simply put, EMPD is a rare skin condition. Sometimes it can be associated with other types of cancer. But that's not always the case. It's a condition where your skin becomes red, scaly, or crusty and grows larger. Sometimes it can go undiagnosed for years after the disease begins.
It is named "Paget's Disease" after Sir James Paget, a brilliant 19th-century British physician. His name has been applied to several other types of cancer because of his contributions to the field of pathology. The word "extramammary" means "outside the breast." This is not to be confused with Paget's disease of the breast.
It is estimated that between 7% and 40% of people with Extramammary Paget's Disease may have an underlying cancer . However, as I mentioned earlier, EMPD is not always a sign of another disease. Early treatment can help reduce your symptoms and help detect underlying cancer.
What are the main types of EMPDs?
There are two main types of EMPDs:
1. Primary EMPD: This is a skin condition that occurs spontaneously. It starts on the skin without any other cause.
2. Secondary EMPD: This is caused by an underlying cancer elsewhere in the body.
Who is more likely to develop EMPD?
Anyone can develop EMPD, but it is more common in the following groups:
- People between the ages of 50 and 80 .
- Common among women .
- It can also be seen among Asian men .
- White people.
But it is important to remember that this is a very rare condition.
Why does this EMPD occur?
What causes primary EMPD?
To be honest, doctors still don't know exactly what causes primary EMPD. They suspect that it may start in certain types of cells in our bodies. These types of cells include:
- Apocrine gland duct cells: These are sweat glands and are located near hair follicles.
- Keratinocyte stem cells: These are cells that help repair the skin.
- Toker cells: These are cells found in the skin (`vulvar tissue`) around the nipple and vaginal opening.
What causes secondary EMPD?
Secondary EMPD is caused by cancer elsewhere in the body. Rectal cancer is said to be the cause of about a third of EMPD cases. In addition, there are other types of cancer that can cause secondary EMPD:
- Anal cancer
- Bladder cancer
- Cervical cancer
- Ovarian cancer
- Prostate cancer
- Uterine cancer
What are the symptoms of EMPD?
The main symptom of EMPD is a skin rash that looks like eczema . Research has shown that in about 65% of EMPD patients, it occurs around the vulva , and in about 20%, it occurs around the anus .
You may see areas on your skin that have these characteristics:
- Like a crust (`Crusty`)
- Turned red (`Red`)
- Scaly
The spots gradually enlarge, and may have irregular edges. As EMPD progresses, deep ulcers or nodules may develop. Other symptoms include:
- Burning sensation
- The skin is red and feels irritated .
- Itchy
- Painful or tender
- Swollen (edema) skin
EMPD most commonly occurs in areas where your sweat glands (especially the apocrine skin glands) are located. It is most commonly seen around the vaginal opening. It can also occur in these areas:
- Around the anus (`Anus`)
- Penis
- Perineum
- Scrotum
Very rarely, EMPD can also be seen in other parts of the body, such as:
- Abdomen
- Armpit
- Bellybutton
- Buttocks
- Ear, eyelid, face or scalp
- Lower chest
- Knee or thigh
It is important to know that about 10% of people with extramammary Paget's disease do not experience any symptoms .
How is this EMPD condition diagnosed?
First, a doctor will ask you about your symptoms and do a physical exam. If EMPD is suspected, they will do a skin biopsy . A biopsy involves taking a small sample of your skin and examining it under a microscope.
When looking under a microscope, a pathologist looks for special, large, abnormal cells called "Paget cells." They also identify these cells by looking at how they react to various chemicals (immunohistochemistry).
How do you find out if you have cancer with EMPD?
After you've been diagnosed with EMPD, your doctor will do several other tests to check for underlying cancer. These tests may include:
- Ultrasound scan (`Ultrasound`)
- Breast examination (mammogram)
- Pap smear
- Colonoscopy or proctoscopy
- CT scan
- Cystoscopy (examination of the bladder)
- Prostate exam
- Retrograde pyelogram (examination of the kidneys and urinary tract)
- Ultrasound or needle biopsy of the lymph nodes
- Upper endoscopy (examination of the throat, esophagus, and stomach)
What are the treatments for EMPD?
Doctors usually treat extramammary Paget's disease with surgery.
- Mohs surgery: In this procedure, the surgeon removes the affected skin layer by layer, preserving the surrounding healthy skin.
- Wide local excision: In this procedure, the surgeon removes the area of EMPD along with some healthy tissue around it. The edges of the wound are then sutured.
Sometimes surgeons have to remove a large area of skin and other tissue. Some people with EMPD around the vulva may need to have part or all of the vulva removed (a `vulvectomy`).
Consider this: If you are unable to undergo surgery due to another health condition or poor health, your doctor may suggest other treatments such as:
- Immunotherapy
- Laser skin resurfacing
- Photodynamic therapy (PDT)
- Radiation therapy
- Topical medications used to treat cancer (cytotoxic drugs)
The most important thing is that if there is an underlying cancer, it must also be treated separately.
Is there a way to reduce the risk of developing EMPD?
Unfortunately, there is nothing we can do to reduce the risk of developing EMPD.
What kind of future can someone with EMPD expect?
After treatment for EMPD, you will need to have follow-up visits with your doctor. This will depend on where the EMPD was. It is common for EMPD to recur in or around the same area where it was previously treated. Therefore, it is very important to continue to follow your doctor's instructions.
Extramammary Paget's disease is very rare in which it spreads (metastasizes) to other parts of the body. However, it can sometimes spread to the lymph nodes or other organs. Your doctor can best explain what is possible in your case.
Can EMPD come back after it has healed?
Yes, EMPD is a condition that can recur even after treatment and recovery, even after many years. Sometimes it can come back after more than 15 years. That's why doctors recommend long-term monitoring and regular checkups.
If I have EMPD, how do I take care of myself?
The best thing you can do is to keep going to your doctor for regular follow-up appointments. If your doctor sees signs of EMPD coming back, they can start treatment right away.
When should I see a doctor?
If you notice any new signs or symptoms of EMPD, see a doctor immediately.
It's normal to feel scared, embarrassed, and frustrated when you notice a skin rash or lump in a sensitive area of your body, such as your genitals. This condition, called Extramammary Paget's Disease (EMPD), can sometimes be confused with other skin conditions, so it can take a while to get an accurate diagnosis. If there is underlying cancer, EMPD can be even more serious.
However, the good news is that most people with EMPD live normal, full lives after treatment. EMPD is usually treated with surgery. If there is underlying cancer, that will also need to be treated. Since Extramammary Paget's Disease can recur, regular checkups with your doctor can help detect it early if it comes back.
The most important things for you to remember (Take-Home Message)
- EMPD is a rare skin condition that may be linked to cancer.
- Pay attention to any red, scaly, crusty, or raised patches of skin, especially around the genitals, armpits, or anus. Symptoms may include itching, burning, and pain.
- If you see a sign like this , don't be shy or afraid, but see a doctor immediately.
- EMPD is diagnosed with a skin biopsy.
- Surgery is the most common treatment.
- It is essential to continue medical checkups, as EMPD can recur.
- Don't worry, most people with EMPD live well after treatment. The key is to get diagnosed early and get the right treatment.
If you have any further questions about this, talk to your doctor. He/she can give you advice that is right for you.
` Extramammary Paget's Disease, EMPD, skin diseases, skin cancer, Paget cells, skin spots, itching, vulva, perianal area, skin diseases, Mohs surgery
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