Eosinophilic Fasciitis

Eosinophilic Fasciitis

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Eosinophilic Fasciitis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Shulman syndrome

Disorder Subdivisions

  • None

General Discussion

Eosinophilic fasciitis is a rare disorder characterized by inflammation of the tough band of fibrous tissue beneath the skin (fascia). The arms and legs are most often affected. Inflammation is caused by the abnormal accumulation of certain white blood cells including eosinophils in the fascia. Eosinophilic fasciitis eventually causes the skin to swell and slowly thicken and harden (induration). The disorder most commonly affects middle-aged adults. The exact cause of eosinophilic fasciitis is unknown. Some researchers believe that eosinophilic fasciitis is a variant of scleroderma (systemic sclerosis), an autoimmune connective tissue disorder characterized by hardening of the skin.

Symptoms

Initial symptoms associated with eosinophilic fasciitis include pain, swelling and inflammation of the skin, especially of the arms and legs. The arms and forearms are affected more often that the legs and thighs. The hands and feet are usually unaffected. Affected skin may become reddened and warm and gradually thicken and harden (induration). Eventually, the skin may lose its elasticity and develop a characteristic woody, puckered, or orange peel texture. These progressive skin changes can potentially limit the mobility of the arms and legs. In some cases, the arms and legs may become stuck in unusual positions (contractures). Other areas that may be rarely affected by eosinophilic fasciitis include the face, abdomen, buttocks, and chest.



Some affected individuals may develop nonspecific symptoms including fatigue, weight loss, fever, and a general feeling of ill health (malaise). Although muscle strength is usually unaffected, muscle pain (myalgia) and inflammation of the joints (arthritis) often occurs. In many cases, an episode of eosinophilic fasciitis follows strenuous physical exercise or activity. Onset is often sudden and the disorder may progress rapidly.



Some cases of eosinophilic fasciitis may be associated with carpal tunnel syndrome, a condition caused by compression of peripheral nerves affecting one or both hands. It is characterized by a sensation of numbness, tingling, burning and/or pain in the hand and wrist. Symptoms are slowly progressive and may eventually make it difficult to form a fist or grasp small objects.



The internal organs (viscera) may be affected in some cases, although only mildly. Blood (hematological) abnormalities have been reported including low levels of circulating red blood cells (anemia) and low levels of circulating platelets (thrombocytopenia). Anemia may cause tiredness, increased need for sleep, weakness, lightheadedness, dizziness, irritability, headaches, pale skin color, and difficulty breathing. Thrombocytopenia may cause individuals to be more susceptible to excessive bruising following minimal injury and to spontaneous bleeding from the mucous membranes, especially those of the gums and nose. Some affected individuals may eventually develop a serious condition known as acquired aplastic anemia. (For more information on this disorder, choose "aplastic anemia" as your search term in the Rare Disorders Database.)

Causes

The exact cause of eosinophilic fasciitis is unknown (idiopathic). Some researchers believe autoimmunity plays a role in the development of the disorder. Autoimmune disorders are caused when the body's natural defenses (antibodies, lymphocytes, etc) against invading organisms suddenly begin to attack perfectly healthy tissue. Researchers have also speculated that exposure to certain toxins, drugs, or other environmental factors including infectious agents (Borrelia burgdorferi) may play a role in the development of eosinophilic fasciitis.



Some scientists believe that eosinophilic fasciitis may be a variant of scleroderma or morphea (localized scleroderma). These rare disorders are characterized by the hardening and thickening of skin and surrounding tissue, often due to the malfunction of the immune system.



Some cases of eosinophilic fasciitis have been related to the ingestion of a dietary supplement known as L-tryptophan. Tryptophan is an essential amino acid found in numerous foods including poultry. L-tryptophan was a dietary supplemental formerly available in the United States as a sleep aid or to treat depression. L-tryptophan was linked to an outbreak of a similar disorder known as eosinophilia-myalgia syndrome in the 1989. (For more information on scleroderma, morphea or eosinophilia-myalgia syndrome, see the Related Disorders section of this report.)



Some cases may be linked to infection with spiral-shaped bacterium (spirochete) known as Borrelia burgdorferi, the same bacterium that causes Lyme disease.



Some researchers believe eosinophilic fasciitis is one of several disorders that should be grouped under the designation fasciitis-panniculitis syndrome (FPS). Disorders under this designation are characterized by hardening and thickening of the skin due to inflammation and fibrosis.

Affected Populations

Eosinophilic fasciitis affects both sexes with consistently reported and significant predominance of one sex over the other The disorder can occur at any age, but most often occurs in individuals between 30-60 years of age. It occurs with greater frequency in Caucasians. Eosinophilic fasciitis was first described in the medical literature in 1974.

Standard Therapies

Diagnosis

A diagnosis of eosinophilic fasciitis is suspected based upon a detailed patient history, a thorough clinical evaluation and laboratory studies such as blood tests that reveal elevated levels of eosinophils in the blood (eosinophilia) or increased levels of certain proteins (immunoglobulins), which are used by the immune system to destroy foreign or invading substances in the body. In some cases, an erythrocyte sedimentation rate (ESR) test may be performed. An ESR test measures the rate at which red blood cells settle in a tube of anticoagulated blood. An elevated ESR indicates that inflammation is present.



A diagnosis of eosinophilic fasciitis is confirmed by surgical removal and microscopic evaluation (biopsy) of affected tissue. A biopsy demonstrates thickening and inflammation of fascia and surrounding tissue.



Treatment

The treatment of eosinophilic fasciitis is directed toward preventing and alleviating tissue inflammation. In some cases, affected individuals improve without treatment (spontaneous remission). Many individuals respond favorably to corticosteroid therapy, and the drug prednisone is often prescribed. Prednisone therapy may be required for two months or longer. In many cases, high doses of corticosteroids started are used at first and slowly tapered off. Cases have been reported where eosinophilic fasciitis eventually recurred following corticosteroid therapy.



Additional treatment is symptomatic and supportive. Surgical decompression of affected nerves may be used to treat carpal tunnel syndrome. Non-steroidal anti-inflammatories (NSAIDs) may be used to provide relief as well.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Li JY. Eosinophilic Fasciitis. NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:8.



JOURNAL ARTICLES

Lamloum M, Sioud DA, Ben Ghorbel I, et al., Shulman syndrome. A report of 4 cases and review of the literature. Tunis Med. 2006;84:189-94.



Carneiro S, Brotas A, Lamy F, et al., Eosinophilic fasciitis (Shulman syndrome). Cutis. 2005;75:228-32.



Antic M, Lautenschlager S, Itin PH. Eosinophilic fasciitis 30 years after - what do we really know? Report of 11 patients and review of the literature. Dermatology. 2005;213:93-101.



Selva O'Callaghan A, Simeon Aznar CP, Sanjurjo Golpe E, Garcia-Patos V, Villardell Tarres M. Eosinophilic fasciitis: analysis of a series of 10 patients. Med Clin (Barc). 2005;125:145-8.



Bukiej A, Dropinski J, Dyduch G, Szczeklik A. Eosinophilic fasciitis successfully treated with cyclosporine. Clin Rheumatol. 2005;24:634-6.



Wojas-Pele A, Wielowieyska-Szybinska D, Lipko-Godlewska S. Eosinophilic fasciitis -current database. Pol Merkuriusz Lek. 2004;16:585-8.



Saez Barcelona JA. Eosinophilic fasciitis and related diseases. Ann Med Interna. 1999;16:477-83.



Varga J, Kahari VM. Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders. Curr Opin Rheumatol. 1997;9:562-70.



Achurra AF, Mendieta MC, Llerena JM, Benitez R. Eosinophilic fasciitis. A report of a clinical case and the current concepts for its diagnosis and treatment. Rev Med Panama. 1997;22:39-44.



Granter SR, Barnhill RL, Duray PH. Borrelial fasciitis: diffuse fasciitis and peripheral eosinophilia associated with Borrelia infection. Am J Dermatopathol. 1996;18:465-73.



Grigoris I, de Launey W, Jevtic AP. Eosinophilic fasciitis associated with L-tryptophan ingestion. Med J Aust. 1992;157:329-30.



FROM THE INTERNET

Graham BS. Eosinophilic Fasciitis. Emedicine Journal, May 3, 2005. Available at: http://www.emedicine.com/DERM/topic119.htm Accessed on: September 30, 2006.



Nasef S. Eosinophilic Fasciitis. Emedicine Journal, January 26, 2006. Available at: http://www.emedicine.com/med/topic686.htm Accessed on: September 30, 2006.

Resources

American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Ave.

Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



Centers for Disease Control and Prevention

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Atlanta, GA 30333

Tel: (404)639-3534

Tel: (800)232-4636

TDD: (888)232-6348

Email: cdcinfo@cdc.gov

Internet: http://www.cdc.gov/



NIH/National Institute of Allergy and Infectious Diseases

Office of Communications and Government Relations

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Tel: (301)496-5717

Fax: (301)402-3573

Tel: (866)284-4107

TDD: (800)877-8339

Email: ocpostoffice@niaid.nih.gov

Internet: http://www.niaid.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



International Scleroderma Network

7455 France Ave So #266

Edina, MN 55435-4702

Tel: (952)583-5735

Tel: (800)564-7099

Email: isn@sclero.org

Internet: http://www.sclero.org



Campaign Urging Research for Eosinophilic Disease (CURED)

PO Box 32

Lincolnshire, IL 60069

Tel: (847)361-3292

Email: ellyn@curedfoundation.org

Internet: http://www.curedfoundation.org



American Partnership for Eosinophilic Disorders

PO Box 29545

Atlanta, GA 30359

Tel: (713)493-7749

Fax: (713)493-7749

Internet: http://www.apfed.org



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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