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Eosinophilic fasciitis

Definition

Eosinophilic fasciitis is a very rare 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 in appearance to scleroderma but is not related.

Causes, incidence, and risk factors

The cause of eosinophilic fasciitis is unknown. Fewer than 300 cases have been reported in the past 35 years.

People with this condition have a buildup of eosinophils, a type of white blood cell, in the affected fascia and muscles. Eosinophils are related to allergic reactions, but their exact function is unknown.

The syndrome affects people of any age, but is more common in people ages 30 to 60.

Symptoms

  • Bone pain or tenderness
  • Carpal tunnel syndrome
    • Numbness or tingling in one or both hands
    • Pain in one or both hands
    • Weakness in one or both hands
  • 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:

Treatment

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

If blood diseases develop, they are treated based on their type.

Expectations (prognosis)

In most cases, the condition goes away within 3 to 5 years. However, symptoms may return (recur) or persist. The outlook is much worse if blood diseases occur.

Complications

Arthritis is a rare complication of eosinophilic fasciitis. Some patients may develop very serious blood disorders or blood-related cancers, such as aplastic anemia or leukemia.

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: 2/7/2011
Reviewed By: Michael E. Makover, MD, professor and attending in Rheumatology at the New York University Medical Center, New York, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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