Eosinophilic fasciitis, or Shulman syndrome, is a rare condition that causes a painful thickening and stiffness in the connective tissue underneath the skin. This is known as the fascia.
The condition most often affects the arms and legs. It develops due to a buildup of white blood cells (eosinophils) in the fascia during the inflammation reaction of the immune system.
Some medical researchers suggest that eosinophilic fasciitis is related to scleroderma, which is an autoimmune condition that causes hard skin.
Read on to learn more about the symptoms and causes of eosinophilic fasciitis. This article also looks at diagnosis, treatment options, possible complications, and more.
Symptoms of eosinophilic fasciitis can include:
- redness, pain, and swelling in both forearms and lower legs
- peau d’orange, or uneven skin that looks like the skin of an orange
- gradual hardening of the skin
- “groove sign,” which refers to the development of grooves over surface veins that become more visible when a person raises the affected limb
- fever
- fatigue
- joint pain
- unexplained weight loss
Symptoms may not affect the face, hands, or feet, though they may extend to the thighs and upper arms. The condition generally does not affect internal organs, unlike scleroderma.
The symptoms of eosinophilic fasciitis vary greatly between individuals. However, it usually develops 7–14 days after intense exercise or physical activity. In rare cases, the condition sets in gradually.
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Research into the causes of eosinophilic fasciitis is ongoing. However, researchers believe that the immune system might play a key role in its development.
The fascia includes all soft tissues made from collagen and usually supports muscle
Eosinophils possibly release an inflammatory protein, or cytokine, known as transforming growth factor-beta (TGF-beta). This may release several different substances that increase scarring in the fascia tissue.
Possible triggers of experiencing eosinophilic fasciitis include:
- unusually intense physical activity or exercise, which is the reported trigger for 50% of people with eosinophilic fasciitis
- insect bites
- radiation therapy
- taking medications such as atorvastatin, carbidopa, and immune checkpoint inhibitors
- cancers, including leukemia and myeloproliferative disorder
- graft-versus-host disease, in which the immune system rejects a graft
To assist in diagnosing eosinophilic fasciitis, a doctor may first rule out scleroderma by checking for “groove sign” and ruling out the involvement of the fingers, face, and hands.
They can also carry out the following tests to assess whether a person has eosinophilic fasciitis:
- Blood tests: A blood test can help show whether a person has extremely high levels of eosinophils early in the condition.
- Skin biopsy: A doctor can confirm the diagnosis by taking a deep skin sample and sending it to a lab for analysis under a microscope.
- MRI scans: These scans can help doctors find any changes to the thickness of the fascia.
The doctor can explain the tests they order and answer any questions a person might have.
Eosinophilic fasciitis treatment aims to lower fascia inflammation and help prevent further symptoms.
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Medications
Doctors usually recommend a course of corticosteroid medications for people who require treatment.
Prednisone is the most common corticosteroid doctors prescribe for this condition. They often start by prescribing a high dose and reduce this over time. A person with eosinophilic fasciitis may need at least 2 months of prednisone treatment.
Nonsteroidal anti-inflammatory drugs (NSAIDs) may also help relieve swelling, stiffness, and discomfort.
Other treatments
Several approaches can help relieve the symptoms, hardening, and mobility issues that might accompany eosinophilic fasciitis.
Surgery might be necessary to relieve the compression of nerves to treat carpal tunnel syndrome or address muscle deformities.
Physical therapy may also restore some motion and flexibility to the areas affected by eosinophilic fasciitis.
A doctor can discuss treatment options and help create a suitable treatment plan. They can also recommend treatments for any complications that develop.
Without treatment, eosinophilic fasciitis can lead to several complications, such as:
- limited movement at the joints due to contractures
- symmetrical nonerosive arthritis, which is a type of joint inflammation
- carpal tunnel syndrome, which results from the fascia pressing on the median nerve that runs from the forearms to the palms
- compartment syndrome, which develops due to increased pressure in certain muscles
Some people with eosinophilic fasciitis may also develop blood disorders, including:
- aplastic anemia
- thrombocytopenia
- lymphoma
Most people who need to take immune system suppressants to treat eosinophilic fasciitis may see their symptoms go away within
If symptoms go away without treatment, the condition might resolve on its own within 2–5 years.
Eosinophilic fasciitis is extremely rare.
According to a 2016 report by the National Organization for Rare Disorders (NORD), fewer than 300 people have received a diagnosis of eosinophilic fasciitis in the medical literature. The condition is most common in adults ages between 30 years and 60 years, but it can occur at any age.
Researchers have not yet found a way to prevent eosinophilic fasciitis, as the underlying cause is still not clear.
Here are some frequently asked questions about eosinophilic fasciitis.
Is eosinophilic fasciitis an autoimmune disease?
Eosinophilic fasciitis may involve the immune system, but researchers rarely describe it as an autoimmune disease due to the unknown nature of its causes. However, according to the NORD, some medical practitioners believe it may be related to an autoimmune condition called scleroderma.
How long does eosinophilic fasciitis last?
For some people, eosinophilic fasciitis may heal without treatment in
What are the first symptoms of eosinophilic fasciitis?
The first symptoms of eosinophilic fasciitis can include:
- pain
- swelling
- inflammation
- tenderness
These symptoms usually affect the arms and forearms but can also affect the legs and thighs.
How long does it take to recover from eosinophilic fasciitis?
Recovering from eosinophilic fasciitis can take up to 5 years without treatment and 3 years with medication. It is possible to make a full recovery.
Eosinophilic fasciitis is a very rare condition that leads to inflammation of the fascia. It can cause pain, swelling, and hard skin in the arms and legs. The condition’s trademark symptom is the “groove sign.”
The exact cause is unclear, but the condition has links to the immune system.
Diagnosis often shows a buildup of white blood cells in blood tests and a thickening of the fascia on MRI scans. Treating the condition typically involves prednisone and other immunosuppressants, which often help resolve the condition within 1–3 years. Eosinophilic fasciitis sometimes resolves without treatment in 2–5 years.