Eosinophilic fasciitis

Eosinophilic fasciitis

Definition:

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 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
- 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
- nails and skin of hands may be dry and shiny

Signs and tests:

erythrocyte sedimentation rate, elevated
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 (rare)

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.

Review Date: 12/1/2001
Reviewed By: Birgit Kovacs, M.D., Division of Rheumatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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