Meige Disease

What is Meige disease?

Meige disease (lymphedema praecox) is a rare form of lymphedema that usually appears between puberty and your mid-20s. Lymphedema praecox is a genetic condition in which your lymphatic system doesn’t develop normally in certain parts of your body. Without the typical lymph nodes and channels, lymphatic fluid builds up instead of going where it should. This causes swelling.

Because Meige disease gets worse over time, a prompt diagnosis and early treatment are best. Many people find relief from treatments that don’t involve surgery.

How common is this condition?

Meige disease is the most common type of primary lymphedema. (Primary means another condition didn’t cause it.) But it’s still a rare disease. To understand what this means, picture one of America’s largest college football stadiums filled with people under age 20. Now imagine one person in it might have primary lymphedema (1 in 100,000 people).

It’s more common in people who are assigned female at birth (AFAB).

What are the symptoms of Meige disease?

Meige disease symptoms include swelling (edema) in your feet, ankles and legs. This swelling, which may appear at puberty, develops slowly over several months. It doesn’t cause pain, but the affected area may feel heavy.

People with lymphedema praecox may also have:

• Hearing loss
• Extra eyelashes (distichiasis)
• Bones in your spine that didn’t form properly
• Blood vessels in your brain that didn’t form correctly

Meige disease is different from Milroy’s disease, which is another form of lymphedema. People with Milroy’s disease have leg swelling in their first year of life. Meige disease shows up years later.

What causes Meige disease?

In people with Meige disease, something goes wrong when their lymphatic system is forming. Researchers have found some genetic variations linked to Meige disease. But many people with this condition don’t have an identified genetic link to it.

It only takes one parent with the variant to pass this on to their child. But not all children will experience the condition.

What are the complications?

Complications of Meige disease may include:

• Cellulitis or another type of skin infection in your legs
• Papillomatosis (wart-like growths)
• Hyperkeratosis (thickened skin)
• Lymphangitis (inflamed lymph vessels)
• Breakdown of skin

Rarely, lymphedema praecox can become cancerous.

How is it diagnosed?

To make a Meige disease diagnosis, a healthcare provider will:

• Perform a physical exam
• Talk with you about your medical history and that of your biological family
• Order one or more tests

Tests a provider may use to diagnose Meige disease lymphedema include:

• Ultrasound
• Magnetic resonance imaging (MRI)
• Lymphoscintigraphy
• Computed tomography (CT)

How is Meige disease treated?

Although there isn’t a cure for Meige disease, treatments help drain lymphatic fluid.

Meige disease treatment includes:

• Compression stockings or devices
• Physical activity, like swimming
• Lymphatic drainage massage
• Leg elevation
• Laser therapy
• Liposuction
• Surgery

What can I expect if I have Meige disease?

Lymphedema praecox is chronic (long-term), so you’ll need to manage it throughout your life. The disease tends to get worse slowly over time. But most people get good results without having surgery. You may need surgery if other treatments don’t work for you.

How do I take care of myself?

You can take care of yourself in several ways. You can:

• Protect your skin from wounds by using moisturizer and wearing shoes outside
• Reduce how much salt you eat to help lessen how much fluid your body holds onto
• Replace compression socks or sleeves every three to six months so they keep working well

When should I see my healthcare provider?

You may have frequent appointments at first. But in the long term, you may only see your provider once a year with Meige disease.

Regular visits with your provider can allow them to monitor how you’re doing. They can see if your condition is getting worse or if the treatment you’re using is working.

You should also contact your provider if you see signs of cellulitis or other complications.

What questions should I ask my healthcare provider?

You may want to ask your provider:

• What’s the best treatment for me?
• How many hours a day do I need to wear compression garments?
• Who in my family may also get this disease?
• How much physical activity do I need?