Eosinophilic fasciitis

Definition

Eosinophilic fasciitis is a syndrome in which muscle tissue underneath the skin, called fascia, becomes inflammed and thickens. 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. There is a gathering 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 precipitated 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

Signs and tests

• Erythrocyte sedimentation rate (ESR ) may be elevated.
• Blood tests may show a high concentration of gamma globulins (a type of immune-system cells) and eosinophils.
• Muscle biopsy, including fascia tissue.
• Skin biopsy, including fascia tissue.

Treatment

Treatment with corticosteroid medications provides relief of the symptoms, especially when it is started early in the disease course. Non-steroids anti-inflammatories (NSAIDs) may also help relief symptoms.

Expectations (prognosis)

Most people have a resolution of this illness within 3 to 5 years. Some recurrences of the illness or persistent disease have been noted.

Complications

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

Calling your health care provider

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.

Illustrations

Superficial anterior muscles

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