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

Eosinophilic fasciitis (pronounced /ˌi.ə.sɪn.əˈfiˌlɪk ˌfæ.ʃiˈɑɪ̯.tɪs/), 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

Classification

Pathophysiology

Causes

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

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

For more information on differentiating eosinophilic fasciitis, click here.

Epidemiology and Demographic

Age

Gender

Race

Risk Factors

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

Natural History, Complications and Prognosis

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

Diagnostic Criteria

Symptoms

  • 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

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

Laboratory Findings

Tests that may be done include:

Imaging Findings

Other Diagnostic Findings

Treatment

Medical Therapy

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

Surgery

Prevention

Related Chapters

References

  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.
  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.
  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.



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