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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

The cause of eosinophilic fasciitis is unknown. Persons 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

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

Exams and Tests

Tests that may be done include:

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

Treatment

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

Outlook (Prognosis)

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

Possible Complications

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

When to Contact a Medical Professional

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

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.

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