Eosinophilic Fasciitis

What is eosinophilic fasciitis?

Eosinophilic fasciitis is a condition that causes your fascia (the layer of tissue under your skin that covers your muscles) to swell and thicken quickly. It’s a type of autoimmune disease, which means your immune system accidentally attacks your body instead of protecting it. If it’s not treated or diagnosed quickly, eosinophilic fasciitis can cause chronic skin changes and limited joint range of motion.

Eosinophilic fasciitis can cause inflammation in fascia located anywhere in your body, but it most commonly affects your arms or legs. Most people notice symptoms such as pain or swelling after intense physical activity.

Eosinophilic fasciitis shares a lot of symptoms with other more common conditions. It’s important to visit your healthcare provider as soon as you notice any new symptoms in your skin, muscles or joints. You may need to visit a rheumatologist, a doctor who specializes in treating inflammatory diseases.

There’s no cure for eosinophilic fasciitis, but most cases go away (go into remission) in a few years. It sometimes comes back in people who have had it once.

Eosinophilic fasciitis vs scleroderma

Both eosinophilic fasciitis and scleroderma are rare autoimmune diseases that cause tissue in your body to thicken. The difference is which kinds of tissues are affected.

Eosinophilic fasciitis affects your fascia, the stretchy layer of connective tissue under your skin. Over time, your fascia thickens and expands, which can make it difficult to move a part of your body.

If you have scleroderma, your immune system triggers other cells to produce too much collagen (a protein). This extra collagen is deposited in your skin and organs, which makes them harden and thicken, similarly to how scars form. But instead of healing over a wound, the extra collagen can damage healthy tissue.

Eosinophilic fasciitis and COVID-19

People with autoimmune diseases — including eosinophilic fasciitis — are at a higher risk of contracting COVID-19 (coronavirus).

Experts haven’t been able to definitively say if having eosinophilic fasciitis makes you more likely to have a severe case of COVID-19. Follow current Centers for Disease Control and Prevention (CDC) recommendations to lower your risk of infection.

Who does eosinophilic fasciitis affect?

Eosinophilic fasciitis can affect anyone, but it most commonly affects people between 30 and 60 years old.

People with certain health conditions may be more likely to develop eosinophilic fasciitis. These conditions include:

• Aplastic anemia.
• Hemolytic anemia.
• Lymphoma.
• Leukemia.
• Certain forms of thyroid disease.

How common is eosinophilic fasciitis?

Eosinophilic fasciitis is extremely rare. There have only been about 300 cases of eosinophilic fasciitis reported in scientific journals. It may be under recognized and underreported.

How does eosinophilic fasciitis affect my body?

Eosinophilic fasciitis causes inflammation in your fascia, the stretchy layer of connective tissue below your skin. Fascia covers and protects your muscles and some of your internal organs. It holds your skin and muscles in place and supports them while they move. Fascia is similar to the stretchy netting you might use in the trunk of your car to hold groceries in place on the way home from the store.

Your fascia naturally has some resistance and toughness to make sure your skin and muscles can’t move too far. But if you have eosinophilic fasciitis, your fascia swells and starts to thicken. Eventually, this inflammation can make it hard to move the skin and muscles around it. If it’s not treated, eosinophilic fasciitis can freeze your fascia in place. This is sometimes referred to as contracture.

Instead of your fascia having its usual tension that supports your limbs while it moves, eosinophilic fasciitis makes it painful or difficult to move the affected parts of your body. The most commonly affected parts of your body include:

• Arms (especially the inside — the same side as the palm of your hand).
• Legs (especially the front near your shins).
• Joints or the areas around them.

It’s less common, but eosinophilic fasciitis can also affect skin on your:

• Face.
• Chest.
• Abdomen.

Some people with eosinophilic fasciitis may also develop inflammation of their joints, similar to a condition called rheumatoid arthritis. Uncontrolled joint inflammation can damage the joint cartilage leading to chronic arthritis.

Similarly, if the fascia in your hand or wrist is affected by eosinophilic fasciitis, the inflammation can put pressure on your median nerve and cause carpal tunnel syndrome.

Eosinophilic fasciitis changes (progresses) quickly. It can start noticeably affecting you in as little as a few weeks. Talk to your healthcare provider right away if you notice any symptoms or changes in your body.

What are the symptoms of eosinophilic fasciitis?

Symptoms of eosinophilic fasciitis include:

• Muscle pain.
• Areas of tender skin.
• Inflammation or swelling.
• Carpal tunnel syndrome.
• Arthritis.
• Skin that looks or feels thicker than usual.
• Puckered skin (skin that looks like an orange peel).

What causes eosinophilic fasciitis?

Experts aren’t sure what causes eosinophilic fasciitis. Like lots of autoimmune diseases, it develops suddenly and sometimes randomly.

Some studies have found that autoimmune diseases can be triggered — something happens to you that makes them develop. Some triggers may include certain types of infections or allergic reactions. Experts think using certain medications or recreational drugs can be triggers for autoimmune diseases too.

Some people with eosinophilic fasciitis notice their symptoms for the first time after intense physical activity like running, working out or doing a job that puts a lot of stress on their arms or legs.

Talk to your healthcare provider about any risk factors you may have for developing an autoimmune disease.

How is eosinophilic fasciitis diagnosed?

Your healthcare provider can diagnose eosinophilic fasciitis in a few ways, including:

• A biopsy of your fascia: A biopsy is the medical term for your provider removing a sample of your tissue for examination. They’ll remove a small section of your affected skin or muscle and send it to a laboratory for testing. Biopsies are usually the most reliable way to diagnose eosinophilic fasciitis.
• A blood test: If you have eosinophilic fasciitis, your blood may have elevated levels of blood cells known as eosinophils (this is where the disease’s name comes from). Eosinophilic fasciitis can't be diagnosed with blood tests alone. Biopsy is needed in most cases.
• Imaging tests: MRIs are helpful at identifying inflammation inside your body, including of your fascia and surrounding tissue as seen in eosinophilic fasciitis. Like blood tests, it’s usually not as conclusive as a biopsy at diagnosing eosinophilic fasciitis.

How is eosinophilic fasciitis treated?

It's not common, but your symptoms may improve randomly without any treatment. More commonly, your healthcare provider will treat eosinophilic fasciitis with corticosteroids. Corticosteroids reduce inflammation and lower your immune system’s activity. Prednisone is the most common corticosteroid used to treat eosinophilic fasciitis.

Your provider may use other treatments in addition to corticosteroids, including:

• NSAIDs: Over-the-counter non-steroidal anti-inflammatory drugs (NSAIDs) can help reduce symptoms such as pain. Talk to your provider before taking any NSAIDS if you’re already receiving corticosteroids. Don’t take NSAIDS for more than 10 days in a row without permission from your provider.
• Immune system suppressing medications: Depending on the severity of your condition, your provider may use a medication that suppresses the overactive immune system responsible for eosinophilic fasciitis.
• Physical therapy: Physical therapy can help stretch and loosen your fascia as it tightens. Your provider or physical therapist will give you exercises that are specific to your symptoms.
• Treating other conditions: Treating the symptoms of other conditions like carpal tunnel syndrome or arthritis can make you feel more comfortable.
• Surgery: If the eosinophilic fasciitis advances enough to cause contractures, you may need surgery to release your fascia (or remove part of it). Talk to your provider or surgeon about what to expect.

How can I manage the symptoms of eosinophilic fasciitis?

Make sure to take your medication as often as your healthcare provider says you should (and for as long as they prescribe). If your provider or physical therapist gives you stretches or other exercises, do them as often as they suggest to help stretch your affected fascia.

If you have carpal tunnel syndrome, you may need to wear a brace that holds your wrist in place while you’re sleeping. Wear it as often as your provider instructs.

How soon after treatment will I feel better?

The inflammation caused by eosinophilic fasciitis should get better a few hours after your first dose of corticosteroids. This relief may be temporary, and your symptoms may come back as corticosteroids or NSAIDs start wearing off. Take your medications as often as your provider prescribes them.

How can I prevent eosinophilic fasciitis?

There's no known way to prevent eosinophilic fasciitis. Because experts aren’t sure what triggers or causes autoimmune diseases, there’s no way to know when — or why — you’ll develop one.

What can I expect if I have eosinophilic fasciitis?

If you have eosinophilic fasciitis, you should expect to manage your symptoms for a while. Talk to your healthcare provider about any changes in your symptoms, especially if they’re getting worse.

There’s no cure for eosinophilic fasciitis. Most people have it for a few years before it goes away (enters remission). Some people with eosinophilic fasciitis never have it again, but others have cases that come back later (recur).

Will I need to miss work or school while I’m recovering from eosinophilic fasciitis?

If you can do your job or schoolwork without putting extra stress on your affected fascia, you shouldn’t need to miss work or school while you’re recovering from eosinophilic fasciitis. Your provider will tell you which activities you should avoid while you’re recovering.

Talk to your provider before resuming any intense physical activity like running or working out.

When should I see my healthcare provider?

Visit your healthcare provider as soon as you notice any new symptoms in your arms, legs or joints — especially if you’re experiencing pain or swelling that doesn’t get better after a few days of rest.

Talk to your provider right away if you’ve already been diagnosed with eosinophilic fasciitis and you notice your symptoms getting worse or if a part of your body is beginning to stiffen.

When should I go to the ER?

Go to the emergency room if you suddenly can’t move or use a part of your body.

What questions should I ask my healthcare provider?

• Do I have eosinophilic fasciitis or another condition?
• How quickly will my symptoms progress?
• Which treatments will I need?
• Will I need surgery?
• When can I resume physical activity?

A note from Cleveland Clinic

Eosinophilic fasciitis is extremely rare. Talk to your healthcare provider if you notice new pain in your arms or legs, especially if it’s not going away or getting better after a few days. Eosinophilic fasciitis can progress very quickly, so the sooner you get your symptoms examined, the faster your provider can diagnose what’s causing them. Your provider will work with you to find a combination of corticosteroids and other treatments to manage your eosinophilic fasciitis symptoms.