Eosinophilic fasciitis

Other Names: Shulman syndrome; EF

Eosinophilic fasciitis is a very rare condition in which muscle tissue underneath the skin, called fascia, becomes swollen and thick. Rapid swelling can occur in the hands, arms, legs, and feet. People with this condition have a buildup of eosinophils, a type of white blood cell, in the affected fascia and muscles.

Eosinophilic fasciitis is similar in appearance to scleroderma. However, in contrast with systemic sclerosis, internal organ involvement in eosinophilic fasciitis is generally absent. Some researchers believe that eosinophilic fasciitis may be a variant of morphea (localized scleroderma).

Cause

The cause of EF is unknown. Rare cases have occurred after taking L-tryptophan supplements. In people with this condition, white blood cells, called eosinophils, build up in the muscles and tissues. Eosinophils are linked to allergic reactions. The syndrome is more common in people ages 30 to 60.

Signs and symptoms

Symptoms may include:

• Tenderness and swelling of the skin on the arms, legs, or sometimes the joints (most often on both sides of the body)
• Arthritis
• Carpal tunnel syndrome
• Muscle pain
• Thickened skin that looks puckered

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Acrocyanosis(Persistent blue color of hands, feet, or parts of face)
• Cellulitis(Bacterial infection of skin)
• Eosinophilia(High blood eosinophil count)
• Fatigue(Tired)
• Macule(Flat, discolored area of skin)
• Muscular edema
• Myalgia(Muscle ache)
• Subcutaneous nodule(Firm lump under the skin)

30%-79% of people have these symptoms

• Arthralgia(Joint pain)
• Arthritis(Joint inflammation)

5%-29% of people have these symptoms

• Fasciitis(Inflammation of the fascia)
• Myositis(Muscle inflammation)
• Paresthesia(Pins and needles feeling)
• Weight loss

Diagnosis

Tests that may be done include:

• CBC with differential
• Gamma globulins (a type of immune system protein)
• Erythrocyte sedimentation rate (ESR)
• MRI
• Muscle biopsy
• Skin biopsy (the biopsy needs to include the deep tissue of the fascia)

Treatment

About 10-20% of people with eosinophilic fasciitis recover spontaneously without treatment. For those who do not, glucocorticoids (0.5–1 mg/kg/d), such as prednisone, are the mainstay therapy. Even with treatment, improvement in symptoms can take weeks or months. Glucocorticoids are successful in treating eosionophilic fasciitis in over 70% of cases.

If glucocorticoids are unsuccessful, methotrexate at low doses (15–25 mg once weekly) is probably the most favored second-line treatment, especially in people with reddish to purpleish (morphea-like) skin lesions. Other treatment options include NSAIDs, D-penicillamine, chloroquine, cimetidine, azathioprine, cyclosporin A, infliximab, UVA-1, and bath PUVA. Physical therapy may help improve joint mobility and decrease contractures. Surgical release has been used in some severe cases to manage significant joint contractures.

Prognosis

In most cases, the condition goes away within 1 to 3 years. However, symptoms may last longer or come back.

Possible Complications Arthritis is a rare complication of EF. Some people may develop very serious blood disorders or blood-related cancers, such as aplastic anemia or leukemia. The outlook is much worse if blood diseases occur.

NIH genetic and rare disease info

• NIH info on Eosinophilic fasciitis

Eosinophilic fasciitis is a rare disease.

Eosinophilic fasciitis Resources
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