Eosinophilic fasciitis

Definition
Eosinophilic fasciitis is a syndrome characterized by tenderness and swelling of the extremities caused by inflammation of the fascia and muscles.

Causes, incidence, and risk factors
The cause of eosinophilic fasciitis is unknown. There is an infiltration 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 arms, legs, trunk, and face may be affected by joint contractures, muscle weakness, and changes in the overlying skin. The syndrome in some cases appears to be precipitated by strenuous physical activity. Any age group may be affected, but most people are between 30 and 60 years old.

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       o Weakness in one or both hands       o Numbness or tingling in one or both hands       o Pain in one or both hands       o Inability to make a fist with one or both hands       o Dry, shiny nails and skin of the hands

Signs and tests

     
  • Elevated erythrocyte sedimentation rate  
  • Hypergammaglobulinemia  
  • 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.

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.

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

Prevention
There is no known prevention, because the cause is unknown.

Johns Hopkins patient information

Last revised: December 3, 2012
by Levon Ter-Markosyan, D.M.D.

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