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Eosinophilic fasciitis

Definition

Eosinophilic fasciitis is a syndrome in which muscle tissue underneath the skin, called fascia, becomes swollen and thick. Rapid swelling can occur in the hands, arms, legs, and feet.

The disease is similar to scleroderma.

Causes, incidence, and risk factors

The cause of eosinophilic fasciitis is unknown. People with this condition have a build up of eosinophils, a type of white blood cell, into the affected fascia and muscles. Eosinophils are associated with allergic-type reactions, but their specific function is largely unknown.

The syndrome is most common in people between ages 30 and 60. In some cases, it appears to be triggered by strenuous physical activity.

Symptoms

  • Bone pain or tenderness
  • Carpal tunnel syndrome
    • Dry, shiny nails and skin
    • Inability to make a fist with one or both hands
    • Numbness or tingling in one or both hands
    • Pain in one or both hands
    • Weakness in one or both hands
  • Joint contractures
  • Muscle weakness
  • Tenderness and swelling of the arms and legs (occasionally including joints)
  • Thickened skin with puckered appearance

Signs and tests

Tests that may be done include:

  • Blood tests to check for gamma globulins (a type of immune system cell) and eosinophils
  • Erythrocyte sedimentation rate (ESR)
  • Muscle biopsy
  • Skin biopsy

Treatment

Treatment with corticosteroids and other immune-suppressing medications provides relief of the symptoms, especially when they are started early in the disease. Nonsteroidal anti-inflammatory drugs (NSAIDs) may also help relieve symptoms.

Expectations (prognosis)

In most cases, the condition goes away within 3 to 5 years. However, symptoms may return (recur) or persist.

Complications

Arthritis is a rare complication of eosinophilic fasciitis. Some patients may develop blood-related cancers.

Calling your health care provider

Call your health care provider for an appointment if you have symptoms of this disorder.

Prevention

There is no known prevention.

References

Harris ED. Budd RC, Genovese MC, Firestein GS, Sargent JS, Sledge CB. Kelley's Textbook of Rheumatology. 7th ed. St Louis, MO: WB Saunders;2005:1302-1303.


Review Date: 2/22/2009
Reviewed By: Ariel D. Teitel, MD, MBA, Chief, Division of Rheumatology, St. Vincent’s Hospital, New York, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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