What Is Eosinophilic Fasciitis

Eosinophilic fasciitis is a rare autoimmune disease that causes the band of tissue situated deep under the skin to swell and become thick. This is called the fascia. It can be very similar to other autoimmune diseases that also cause the fascia to swell and thicken.¹

Eosinophilic fasciitis means the band of tissue, the fascia, causes rapid swelling and thickness.² Eosinophilic fasciitis falls into the rare disease category. This condition is so rare only 300 cases have been recorded in medicine.³

Eosinophilic refers to a specific group of white blood cells that are found in our bodies. They help us fight mainly allergies, inflammations, infections and protect us against disease. Fascia is a band of tissue found beneath the skin. This band of tissue connects bones and ligaments, holds our organs in our body together.

• Eosinophilic fasciitis is also known as EF 
• It is where the fascia (the band of tissue) thickens and swells 
• Occurs after strenuous exercise 
• Can be misdiagnosed for other autoimmune diseases 

Overview

Did you know according to the National Institutes of Health (NIH) there are approximately 7,000 rare diseases affecting 25 to 30 million Americans alone? To break this down more, that is 1 in 10 Americans suffering from a rare disease.^4,5 Approximately 80% of rare diseases are estimated to have a genetic origin. A rare disease is a medical condition and are not that common.² Genetic origin is what we are made up of. For example, traits passed down from our parents like eye colour, hair colour, body structure, are you tall like your parents? In science terms, our structure and how our bodies are made up are known as genetics.

Eosinophilic fasciitis (EF) also known as Shulman’s syndrome. The name Shulman comes from the doctor who first discovered the disease in 1974.⁶

Most common body parts affected are the hands, arms, legs and feet. One characteristic people have with EF is strenuous exercise 1-2 weeks before rapid onset. Alongside inflammation in the early stages, redness is present on the skin surface and often pain.^5,6 Other general symptoms can also include high temperature and tiredness.^4,6

People often mistake Eosinophilic fasciitis for Scleroderma. Scleroderma is an autoimmune disease that can cause swelling of the skin. An autoimmune disease refers to when the body attacks itself and fights healthy skin cells instead of protecting them. Eosinophilic fasciitis is also an autoimmune disease, which is why the two often are mistaken for one another.

One way to try to distinguish EF from scleroderma is that EF does not involve fingers, unlike scleroderma, which does. Some medical doctors who research autoimmune diseases believe EF is a variant of systemic sclerosis, which is an autoimmune connective tissue disorder, which has symptoms of hardening of the skin.⁷

Causes of eosinophilic fasciitis

The overall known source of origin is unknown, but intense exercise and trauma are recognised as a known link to the cause.⁸

More rare causes

Patients can also present with other problems, such as:

1. Myopathy, which is a general term used to describe any disease that affects the muscle movement in the body^9,10
2. Peripheral neuropathy, this nerve damage causes numbness, tingling and pain. It often affects the hands and feet¹¹
3. Carpal tunnel syndrome, this common condition causes pain, tingling and numbness in the hand and forearm
4. Inflammatory arthritis or IA is joint inflammation caused by an overactive immune system. It usually affects more than one joint in the body.¹² Your immune system is your body's defence against infections. An example of an overactive immune system would be having an allergic reaction¹³

Signs and symptoms of eosinophilic fasciitis

It is documented in medical literature that it has been difficult to establish the exact characteristics of the disease. This is mainly due to a lack of clinical trials and reported cases. This prevents doctors from developing a clear picture of known symptoms and prognosis. With this in mind, not every person will present with all the same signs and symptoms as defined below:⁸

• Symmetrical swelling to hands, feet, arms or legs
• Redness to the skin
• Pain
• Fever
• Generalised fatigue
• The areas of swelling never reach the fingertips
• Pitting means that when you press your skin, it leaves a fingerprint 

Management and treatment for eosinophilic fasciitis

• Preventing inflammation
• Self-healing
• Corticosteroid therapy – steroids
• Prescribed medication such as prednisolone
• Surgery to treat other linked conditions such as Carpal Tunnel syndrome
• Physiotherapy to improve movement
• Pain relief and anti-inflammatory medication such as Non-steroidal anti-inflammatories (NSAIDS)⁸

Diagnosis

• By consulting with a medical professional, they will conduct a medical history, medical assessment, and lab testing. Some noticeable signs are Venus grooves, which appear as grooves on the surface of the skin. Another sign is when the patient is asked to put their hands together (like a prayer), however, they cannot press the hands flat but the fingers still touch creating a curved shape
• Blood tests can reveal increased levels of proteins in the blood also known as immunoglobulins. These are used to remove bad substances in the body. Blood tests can also show increased levels of eosinophils, which are white blood cells that fight disease
• A biopsy is another way to confirm eosinophilic fasciitis. This is where they take a deep sample of your skin reaching the fascia to test. The doctor will then place the tissue sample under a microscope to determine thickening and inflammation
• MRI which is a magnetic scan can show differences in the fascia tissue, such as thickening

Affected people 

Eosinophilic fasciitis can affect everyone. The medical evidence of confirmed cases suggests women are more affected than men are. The rare disease can occur at any age but amongst the known cases, it commonly affects people between the ages of 30-60 years old.⁸

Patient support groups/external organisations

If you have Eosinophilic Fasciitis, support groups are a great way of connecting with other people who are in a similar situation; talking and connecting with other people can benefit you in any obstacles you may face. 

1. Autoimmune association
2. Autoimmune registry
3. Every life foundation and rare diseases
4. Genetic alliance
5. Global genes
6. National organisation of rare diseases 

FAQs

How can I prevent eosinophilic fasciitis?

Eosinophilic fasciitis is a rare disease. Although there are treatments, there are no known ways to prevent you from getting this condition. Medical professionals do not know the cause, therefore there are no confirmed ways to prevent it. 

How common is eosinophilic fasciitis?

Eosinophilic fasciitis is a very rare disease; only around 300 cases have been reported. However, that is not to say there are more cases. Some may have not been reported or could’ve been misdiagnosed.

When should I see a doctor?

You should see a doctor if you have any of the symptoms in your hands, arms, legs, or feet, or if you notice thickening and inflammation under the skin that doesn’t improve over time. Always seek medical advice if you are concerned about Eosinophilic fasciitis. 

Summary

Eosinophilic fasciitis is a rare disease that affects women more than men. It’s an autoimmune disease that has similar characteristics to scleroderma. The onset of symptoms occurs after strenuous exercise; however, people can present with different symptoms. Corticosteroids are the main treatment for this condition. If you think you may have Eosinophilic fasciitis you should see a doctor.