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Eosinophilic Fasciitis

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Copyright 1991, 1992, 1999, 2007, 2008, 2009, 2013

NORD is very grateful to Gerald J. Gleich, MD, Professor of Dermatology and Medicine, Department of Dermatology, University of Utah, for assistance in the preparation of this report.

Synonyms of Eosinophilic Fasciitis

Disorder Subdivisions

General Discussion

Introduction
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 specific symptoms and severity of eosinophilic fasciitis can vary from one individual to another. The exact cause of eosinophilic fasciitis is unknown.

Introduction
Eosinophilic fasciitis, also known as Shulman syndrome, is named after the physician who, in 1974, was the first to report on the disorder in the medical literature. 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

Although researchers have been able to establish a clear syndrome with characteristic or "core" symptoms, much about eosinophilic fasciitis is not fully understood. Several factors including the small number of identified cases, the lack of large clinical studies, and the possibility of other unknown influencing factors prevent physicians from developing an accurate picture of associated symptoms and prognosis. Therefore, it is important to note that affected individuals may not have all of the symptoms discussed below.

The onset of eosinophilic fasciitis is often sudden (acute), developing over a few days or weeks. In these cases, the disorder may progress rapidly. Less often, a less severe form of disorder that progresses more slowly can develop. In many cases, an episode of eosinophilic fasciitis follows strenuous physical exercise or activity.

Specific symptoms and severity can vary greatly from one individual to another. Both sides of the body are affected in most cases, but rare cases where only one side of the body is affected have also been reported (unilateral eosinophilic fasciitis).

Initial symptoms associated with eosinophilic fasciitis include pain and 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 areas may initially become tender. Affected individuals may develop characteristic shallow grooves or furrows in the skin that run along the paths of underlying veins (venous grooving). Affected skin may become reddened (erythema) 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 joints in the arms and legs may become stuck in unusual positions (contractures). Other areas that may be rarely affected include the face, abdomen, buttocks, and chest. In some cases, localized areas of scleroderma (morphea) may develop.

Some affected individuals may develop nonspecific symptoms including fatigue, weight loss, fever, and a general feeling of ill health (malaise) or a general lack of strength (asthenia). Although muscle strength is usually unaffected, muscle pain (myalgia) and inflammation of the joints (arthritis) often occurs. Bone pain has also been reported.

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.

Causes

The exact cause of eosinophilic fasciitis is unknown (idiopathic). Researchers believe that the disorder results due to a nonspecific triggering event that causes an abnormal immune system response, specifically an abnormal allergic or inflammatory reaction. This abnormal response causes the overproduction and accumulation of eosinophils and other white blood cells in certain tissues of the body. The exact reason for this overproduction and accumulation is not fully understood.

Researchers have speculated that exposure to certain toxins, drugs such as simvastatin or phenytoin, or other environmental factors including infectious agents (Borrelia burgdorferi) may play a role in the development of eosinophilic fasciitis. Such environmental factors may be the underlying cause the abnormal inflammatory response in affected tissue.

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 reports suggest that eosinophilic fasciitis may be associated with extreme physical activity. Some cases developed following strenuous exercise (exercise-induced eosinophilic fasciitis). Trauma has also been associated with the disorder.

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.

In some cases, eosinophilic fasciitis can be associated with another disorder such acquired aplastic anemia, hemolytic anemia, myelodysplastic syndromes, myeloproliferative disorders, lymphoma, leukemia, thyroid disorders, and primary biliary cirrhosis. The relationship between these disorders and eosinophilic fasciitis, if any, is not completely understood. (For more information on these disorders, choose the specific disorder name as your search term in the Rare Disorders Database.)

Affected Populations

Eosinophilic fasciitis affects both sexes. Some reports suggest that women are affected with greater frequency than men. The disorder can occur at any age (with age at diagnosis having ranged from 1-88), but most often occurs in individuals between 30-60 years of age. It occurs with greater frequency in Caucasians. Approximately 300 cases have been reported in the medical literature.

Related Disorders

Symptoms of the following disorders can be similar to those of eosinophilic fasciitis. Comparisons may be useful for a differential diagnosis.

Scleroderma is a rare connective tissue disorder characterized by abnormally increased production and accumulation of collagen, the body's major structural protein, in skin and other organs of the body. There are systemic and localized forms of scleroderma. Systemic scleroderma is characterized by hardening (induration) and thickening of the skin and abnormal degenerative changes and formation of fibrous tissue (fibrosis) in certain organs of the body including the lungs, heart, kidneys, and GI tract. Associated symptoms, which may vary widely from case to case, may include abnormal discoloration of and pain affecting the fingers and toes upon exposure to cold temperatures (Raynaud's phenomenon); abnormal tightness, thickening, "waxiness," and loss of elasticity of the skin; shortness of breath; difficulty swallowing; muscle weakness; joint pain; heart abnormalities including irregular heartbeats (palpitations); kidney (renal) abnormalities; and/or other symptoms and findings. In individuals with localized scleroderma, involvement is restricted to the skin, tissue under the skin (subcutaneous tissue), and, in some cases, underlying muscle and bone. Linear scleroderma is a localized form of scleroderma that may involve only certain areas of the body, such as an arm, a leg, or a portion of the face. It is characterized by multiple lesions of the skin, abnormally increased or decreased skin pigmentation (hyper- or hypopigmentation), and associated atrophy of the skin, subcutaneous tissue, muscle, and bone. Although the exact cause of scleroderma is unknown, researchers suggest that the disorder represents an abnormal autoimmune response. (For further information, choose "scleroderma" as your search term in the Rare Disease Database.)

Morphea, also known as localized scleroderma, is a rare skin disorder characterized by the accumulation of collagen in the skin and subcutaneous tissues. Affected tissue may thickened and harden in response to inflammation caused by collagen accumulation. Individuals with morphea may not have any apparent symptoms (asymptomatic). Skin discoloration and joint pain (arthralgia) may occur in some cases. The exact cause of morphea is unknown, although it is believed to be an autoimmune disorder.

Eosinophilia-myalgia syndrome is a rare disorder associated with the ingestion of large amounts of contaminated L-tryptophan, a dietary supplement often sold in health food stores. The contaminant remains unknown. It is a disease of abrupt onset causing severe, disabling, chronic muscle pain (myalgia); skin symptoms such as inflammation of the tough band of fibrous tissue beneath the skin (fascia); and other neurotoxic reactions. Affected individuals have elevated levels of certain white blood cells (eosinophils) in various tissues of the body (eosinophilia). Diagnosis is not easy and depends on finding unusually high levels of eosinophils (circulating white blood cells) over a period of at least six months. (For more information on this disorder, choose "eosinophilia myalgia" as your search term in the Rare Disease Database.)

Eosinophilic disorder is a general term for any disorder characterized by infiltration of the skin and tissue by a certain type of white blood cell called eosinophils, including disease resulting from arthropod bites, infections, and drug reactions. Churg-Strauss syndrome, hypereosinophilic syndrome and eosinophilic cellulitis are examples of disorders characterized by elevated levels of eosinophils. (For more information on these disorders, choose the specific disorder name as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
A diagnosis of eosinophilic fasciitis is suspected based upon a detailed patient history, a thorough clinical evaluation, and various laboratory studies. Characteristic skin grooves, known as a Prayer sign, is indicative of eosinophilic fasciitis and easily seen upon physical examination.

Clinical Testing and Workup
Laboratory studies can include 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.

Magnetic resonance imaging (MRI) can reveal characteristic changes in the fascia including fascial thickening. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues.

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 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. For example, surgical decompression of affected nerves may be used to treat carpal tunnel syndrome. Surgery may also be recommended to treat contractures. Non-steroidal anti-inflammatories (NSAIDs) may be used to provide relief as well. Physical therapy may be beneficial in some cases.

Investigational Therapies

Additional drugs have been used to treat individuals with eosinophilic fasciitis including drugs that suppress the activity of the immune system (immunosuppressive drugs) such as cyclosporine, cyclophosphamide, or azathioprine. Hydroxychloroquine, rituximab, infliximab, dapsone and immunoglobulins have also been used. These drugs have been used either alone or in combination. More research is necessary to determine the long-term safety and effectiveness of various medications for the treatment of individuals with eosinophilic fasciitis.

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:

Toll-free: (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

For more information about clinical trials conducted in Europe, contact: https://www.clinicaltrialsregister.eu/

Organizations related to Eosinophilic Fasciitis

References

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

JOURNAL ARTICLES
De Masson A, Bouaziz JD, Peffault de Latour R, et al. Severe aplastic anemia associated with eosinophilic fasciitis: report of 4 cases and review of the literature. Medicine (Baltimore). 2013;92:69-81. http://www.ncbi.nlm.nih.gov/pubmed/23429351

Lebeaux D, Frances C, Barete S, et al. Eosinophilic fasciitis (Shulman disease): new insights into the therapeutic management from a series of 34 patients. Rheumatology (Oxford). 2012;51:557-561. http://www.ncbi.nlm.nih.gov/pubmed/22120602

Lebeaux D, Sene D. Eosinophilic fasciitis (Shulman disease). Best Pract Res Clin Rheumatol. 2012;26:449-458. http://www.ncbi.nlm.nih.gov/pubmed/23040360

Samona J. Orthopedic considerations with eosinophilic fasciitis: a case report and literature review. Case Report Orthop. 2012;2012:865360. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3504250/

Servy A, Clerici T, Malines C, et al. Eosinophilic fasciitis: a rare skin sclerosis. Patholog Res Int. 2011;2011:716395. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2995928/

Daniel RS, Lavery S, Maize JC Jr., Brown AN, Bolster MB. Unilateral eosinophilic fasciitis: an under-recognized subtype? J Clin Rheumatol. 2009;15:247-249. http://www.ncbi.nlm.nih.gov/pubmed/19590443

Carneiro S, Brotas A, Lamy F, et al. Eosinophilic fasciitis (Shulman syndrome). Cutis. 2005;75:228-32. http://www.ncbi.nlm.nih.gov/pubmed/15916220

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.
http://www.ncbi.nlm.nih.gov/pubmed/16902285

Trallero Araguas E, Selva O'Callaghan A, Simeon Aznar CP, et al. Eosinophilic fasciitis: analysis of a series of 10 patients. Med Clin (Barc). 2005;125:145-8.
http://www.ncbi.nlm.nih.gov/pubmed/15989857

Bukiej A, Dropinski J, Dyduch G, Szczeklik A. Eosinophilic fasciitis successfully treated with cyclosporine. Clin Rheumatol. 2005;24:634-6. http://www.ncbi.nlm.nih.gov/pubmed/15864687

Wojas-Pelc A, Wielowieyska-Szybinska D, Lipko-Godlewska S. Eosinophilic fasciitis -current database. Pol Merkuriusz Lek. 2004;16:585-8. http://www.ncbi.nlm.nih.gov/pubmed/15510903

Varga J, Kahari VM. Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders. Curr Opin Rheumatol. 1997;9:562-70. http://www.ncbi.nlm.nih.gov/pubmed/9375286

Granter SR, Barnhill RL, Duray PH. Borrelial fasciitis: diffuse fasciitis and peripheral eosinophilia associated with Borrelia infection. Am J Dermatopathol. 1996;18:465-73.
http://www.ncbi.nlm.nih.gov/pubmed/8902092

Grigoris I, de Launey W, Jevtic AP. Eosinophilic fasciitis associated with L-tryptophan ingestion. Med J Aust. 1992;157:329-30. http://www.ncbi.nlm.nih.gov/pubmed/1435477

FROM THE INTERNET
Henning PM, Mount GR, Kortan ND, Nasef S, Lohr KM. Eosinophilic Fasciitis. Emedicine Journal, January 18, 2012. Available at: http://emedicine.medscape.com/article/329515-overview Accessed on: July 24, 2013.

Graham BS. Dermatolgoic Manifestations of Eosinophilic Fasciitis. Emedicine Journal, June 6, 2013. Available at: http://emedicine.medscape.com/article/1065199-overview Accessed on: July 24, 2013.

Report last updated: 2013/09/06 00:00:00 GMT+0