Eosinophilic fasciitis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2] M. Khurram Afzal, MD [3]

Synonyms and keywords: EF; Schulman syndrome

Eosinophilic fasciitis (pronounced Template:IPA), or EF, is a form of fasciitis. It is distinguished from scleroderma primarily because the affected area is the fascia, not the dermis as in scleroderma. Also, unlike scleroderma, Raynaud's phenomenon and telangiectasia are not observed.

It was first described in 1975,^[1] and it is not yet known whether it is actually a distinct condition or just a different presentation. However, it remains used for diagnostic purposes.

It is more common in men than in women.^[2] Several cases have been reported after strenuous exercise.

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.

Eosinophilic fasciitis must be differentiated from other diseases that cause skin thickening and edema such as:

• Scleredema
• Scleroderma
• Scleromyxedema
• Chronic graft-versus-host disease
• Drug induced scleroderma
• Scleroderma overlap syndromes
• Diabetic cheiroarthropathy
• Myxedema
• Nephrogenic systemic fibrosis

For more information on differentiating eosinophilic fasciitis, click here.

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

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. 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.

• 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

Tests that may be done include:

• Gamma globulins (a type of immune system cell)
• Erythrocyte sedimentation rate (ESR)
• MRI
• Muscle biopsy
• Skin biopsy

Common treatments include corticosteroids^[3] such as prednisone, though other medications such as hydroxychloroquine^[4] have also been used.

• Eosinophilia

Template:Diseases of the musculoskeletal system and connective tissue

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