What Is Eosinophilic Fasciitis and How Does It Affect the Body?

Eosinophilic Fasciitis: Diagnosis, Triggers & Management

Eosinophilic fasciitis, or Shulman syndrome, as characterised and described by Dr Lawrence E. Shulman, is a rare autoimmune disease. In this syndrome, your fascia swells and thickens, causing skin changes. The fascia is a tissue present under your skin, covering the muscles. This syndrome mainly happens when your immune system attacks you instead of protecting you.

Building awareness about this rare autoimmune disease is necessary to undergo proper treatment. Read below to learn about its causes, symptoms, and treatment options.

How Does Eosinophilic Fasciitis Affect Your Body?

Eosinophilic fasciitis mainly affects your fascia, which plays a major role in your body. It is a stretchy connective tissue that protects your muscles and organs. Moreover, it also supports muscle and skin movement.

Shulman syndrome inflames and thickens this tissue, making it challenging to move the skin and muscles underneath it. If left untreated, the fascia can freeze in place, causing massive pain and restricted movement.

The syndrome typically affects the arms and feet but can also affect your chest, face, and abdomen. It progresses fast and can lead to joint inflammation and carpal tunnel syndrome.

What Causes Eosinophilic Fasciitis?

Eosinophilic fasciitis occurs when the immune system mistakenly attacks the fascia, the connective tissue beneath the skin. Blood tests in affected individuals often show markers such as peripheral eosinophilia (high eosinophil count) and elevated erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP).

Eosinophils, a type of white blood cell, normally help fight infections and control inflammation. They release a protein called TGF-beta, which activates fibroblasts, responsible for repairing connective tissues.

However, in eosinophilic fasciitis, also known as Shulman syndrome, excessive fibroblast activation leads to the overproduction of type I collagen, TIMP-1, and fibronectin. This causes the fascia to become abnormally thick, hard and tight, resulting in pain and restricted movement.

What are the symptoms of eosinophilic fasciitis?

Shulman syndrome manifests itself by showing symptoms in the following manner:

• Joint stiffness
• Muscle pain
• Thicker skin
• Skin looking like an orange peel
• Inflammation
• Carpal tunnel syndrome
• Fatigue
• Swelling
• Tender skin patches
   

What Triggers Eosinophilic Fasciitis?

Eosinophilic fasciitis symptoms typically emerge after intense physical activity like running. Some common trigger factors of this syndrome include:

• Radiation therapy
• Chronic graft-versus-host disease
• Immune checkpoint inhibitor medications
• infections
   

How do doctors diagnose eosinophilic fasciitis?

If you identify with any of the above symptoms, it is important to consult a doctor immediately. Doctors usually perform the following tests to diagnose this syndrome:

• Fascia Biopsy: Your doctor will remove a small part of the fascia from an affected area (typically the arms) for laboratory testing. They can check for inflammation under a microscope and sample muscle tissue for more accurate results. It is one of the most reliable diagnostic tests for Shulman syndrome.
• Imaging Test: Magnetic resonance imaging (MRI) can detect inflammation inside your body. This helps identify whether your fascia or surrounding tissue is inflamed or abnormal. However, it is not as reliable as a biopsy.
• Blood Test: Eosinophilic fasciitis can increase the amount of eosinophils in your blood. This can help diagnose the disease. However, you may or may not have it. Therefore, a blood test alone is not conclusive of diagnosis.
   

What are the treatment options for eosinophilic fasciitis?

Corticosteroids are the first-line treatment for eosinophilic fasciitis. They are synthetic and prescription medications that can treat the inflammation of your fasciitis. However, this might not be sufficient to treat the syndrome. Therefore, doctors may use the following:

• Immune System Suppressing Medicine: Since it is an autoimmune disease, your doctor may use immune system suppressants. These limit the overactive immune system and suppress the symptoms.
• Non-Steroidal Anti-Inflammatory Drugs: NSAIDs address the syndrome by treating the pain. Using NSAIDs together with corticosteroids significantly increases the risk of gastrointestinal bleeding, ulcers, and perforation. If both medications are necessary, your doctor will likely prescribe an additional medication to protect your stomach and will monitor you closely. Do not take over-the-counter NSAIDs without consulting your doctor if you are on corticosteroid therapy.
• Surgery: Doctors usually recommend surgery if your condition has reached an advanced stage (contractures). In this, your fascia freezes, making movements extremely restricted. Surgery helps in releasing the stiffness in your fascia. In some cases, doctors may remove some part of it.
• Physical Therapy: Apart from medicines and surgery, exercise and stretches can help loosen your fascia. However, these exercises will differ according to different people. A physical therapist usually suggests exercises specific to your symptoms.
   

Experts are yet to find a permanent cure for eosinophilic fasciitis. However, research is ongoing regarding cures and treatments. Living with this condition can be uncomfortable and painful in different ways. It is always crucial to consult a doctor if you experience any symptoms. The syndrome can progress quickly, making prompt medical action necessary.