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

## Overview

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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.

## Historical Perspective

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## Classification

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## Pathophysiology

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## Causes

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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.

## Differentiating Eosinophilic Fasciitis from other Diseases

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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.

## Epidemiology and Demographic

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### Age

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### Gender

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### Race

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## Risk Factors

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

## Natural History, Complications and Prognosis

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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.

## Diagnosis

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### Diagnostic Criteria

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### Symptoms

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* 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

### Physical Examination

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* Tenderness and swelling of the arms and legs (occasionally including joints)
* Thickened skin with puckered appearance

### Laboratory Findings

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Tests that may be done include:

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

### Imaging Findings

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### Other Diagnostic Findings

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## Treatment

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### Medical Therapy

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

### Surgery

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### Prevention

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## Related Chapters

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* Eosinophilia

## References

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1. ↑ Shulman LE (1975). "Diffuse fasciitis with eosinophilia: a new syndrome?". Trans. Assoc. Am. Physicians. 88: 70–86. PMID 1224441.
2. ↑ Wojas-Pelc A, Wielowieyska-Szybińska D, Lipko-Godlewska S (2004). "[Eosinophilic fasciitis--current database]". Pol. Merkur. Lekarski (in Polish). 16 (96): 585–8. PMID 15510903.CS1 maint: Multiple names: authors list (link) CS1 maint: Unrecognized language (link)
3. ↑ Antic M, Lautenschlager S, Itin PH (2006). "Eosinophilic fasciitis 30 years after - what do we really know? Report of 11 patients and review of the literature". Dermatology (Basel). 213 (2): 93–101. doi:10.1159/000093847. PMID 16902285.CS1 maint: Multiple names: authors list (link)
4. ↑ Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB (1988). "Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases". Semin. Arthritis Rheum. 17 (4): 221–31. PMID 3232080.CS1 maint: Multiple names: authors list (link)

Template:Diseases of the musculoskeletal system and connective tissue

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