Eosinophilic Fasciitis

Eosinophilic fasciitis is a rare condition where the fascia that covers your muscles swells and thickens. This leads to symptoms like pain and swelling. Healthcare providers also sometimes call it Shulman syndrome.

Breaking down the condition’s name can help you understand what it is and how it affects you:

• Eosinophilic: Eosinophils are a type of white blood cell. They usually help your immune system protect you against germs or other harmful cells. But if your body makes too eosinophils, they can start damaging you instead. Healthcare providers call this eosinophilia.
• Fasciitis: The fascia is a stretchy layer of connective tissue below your skin. It holds your skin and muscles in place and supports them while they move. It’s kind of like the stretchy netting you might use in the trunk of your car to hold groceries in place on the way home from the store. Fasciitis is the medical term for inflammation in your fascia.

This condition most commonly affects your:

• Arms, especially on the same side as the palm of your hand
• Legs, especially the front near your shin
• Joints or the areas around them

It’s less common, but eosinophilic fasciitis can affect skin on your face, chest and belly, too.

Symptoms of eosinophilic fasciitis

Eosinophilic fasciitis causes painful inflammation in your affected fascia. You may notice symptoms like:

• Swelling or puffiness
• Pain, usually an aching feeling
• Skin that feels thick, firm and tight (some people describe it as feeling “woody”)
• Skin that’s dimpled like an orange peel
• Stiff joints that are hard to move

Symptoms can come on quickly, within a few days. But it can also take weeks for them to become noticeable.

Eosinophilic fasciitis shares lots of symptoms with other, more common conditions. Visit a healthcare provider as soon as you notice any new symptoms in your skin, muscles or joints, especially if it’s hard or painful to move.

Eosinophilic fasciitis causes

Eosinophilic fasciitis is an autoimmune disease. These conditions happen when your immune system damages your body instead of protecting it.

If you have Shulman syndrome, your body makes too many eosinophils. The extra immune cells can turn on your body and start damaging your fascia. Experts aren’t sure what causes this.

Some triggers may include:

• Intense physical activities or exercises, especially if they’re not part of your usual routine
• Injuries
• Infections
• Starting a new medication or a treatment like radiation therapy or dialysis

Risk factors

Anyone can experience Shulman syndrome. But you may be more likely to if you’re between the ages of 40 and 60. That’s when most people are diagnosed.

Having certain other health conditions can increase your risk, including:

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

How doctors diagnose eosinophilic fasciitis

A healthcare provider will diagnose eosinophilic fasciitis with a physical exam and some tests. They’ll examine your body and ask about any symptoms you’ve experienced. Tell your provider when you first noticed pain, swelling or other issues, and if the symptoms seem worse at certain times of day or after any activities.

Your provider can use a few tests to help diagnose Shulman syndrome, including:

• A fascia biopsy: They’ll remove a small sample of your affected skin, fascia or muscles to look at under a microscope. This is the best way to confirm eosinophilic fasciitis.
• Blood tests: These can show extra eosinophils in a sample of your blood. 
• Imaging tests: Your provider can use tests like an MRI to look for inflammation in your body. 

Eosinophilic fasciitis treatments

Your healthcare provider will suggest ways to manage the symptoms you experience. The most common eosinophilic fasciitis treatments include:

• Corticosteroids: These are prescription anti-inflammatory medications. Prednisone is the most common Shulman syndrome medication.
• NSAIDs: Over-the-counter anti-inflammatory medications can relieve pain and reduce inflammation. Your provider will tell you which ones are best for you and how often it’s safe to take them.
• Immunosuppressants: These medications reduce your immune system’s activity. They can treat more severe cases.
• Treatments for specific symptoms: Your provider will help you manage other issues, like carpal tunnel syndrome or arthritis, if eosinophilic fasciitis causes them. They’ll tell you what to expect.
• Surgery: It’s rare to need surgery. But your provider may suggest it if you have severe symptoms that don’t get better with other treatments. A surgeon will cut your fascia to loosen it (this is called “a release”) or remove some of the fascia. This will make it easier to move your affected skin and muscles.

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 have 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 have worsening symptoms or part of your body is stiffening. Go to the emergency room if you suddenly can’t move a body part.

What can I expect if I have this disease?

You should expect to manage the symptoms for a long time. There’s no cure for eosinophilic fasciitis. But you may enter remission. This is a long period between bouts of symptoms. It’s rare, but you may never have symptoms again.