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$Unique_ID{BRK03713}
$Pretitle{}
$Title{Eosinophilic Fasciitis}
$Subject{Eosinophilic Fasciitis Eosinophlic Syndrome Eosinophia Scleroderma
Carpal Tunnel Syndrome}
$Volume{}
$Log{}
Copyright (C) 1991, 1992 National Organization for Rare Disorders, Inc.
814:
Eosinophilic Fasciitis
** IMPORTANT **
It is possible that the main title of the article (Eosinophilic
Fasciitis) is not the name you expected. Please check the SYNONYM listing to
find the alternate names and disorder subdivisions covered by this article:
Synonyms
Eosinophlic Syndrome
Eosinophia
Information on the following diseases can be found in the Related
Disorders section of this report:
Scleroderma
Carpal Tunnel Syndrome
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Eosinophilic Fasciitis is a disorder of unknown cause characterized by
symmetric and painful inflammation and loss of elasticity in the tissues of
the hands, arms, legs and feet. The disorder has been recently recognized as
a variant of Scleroderma, a disease in which connective tissue in the body
shrinks and hardens. Eosinophilic Fasciitis most commonly affects middle-
aged men. It has also been related to ingestion of L-Tryptophan, a food
supplement sold in health food stores as a sleeping aid.
Symptoms
Eosinophilic Fasciitis most commonly occurs in the fourth to sixth
decades of life. Few childhood cases have been reported. The initial
symptoms may include pain, swelling and inflammation of the skin, which
usually takes on a characteristic orange-peel appearance. The arms and
forearms are affected more often than the thighs and legs. Arm and leg
movements gradually become restricted. Inflammation of the tendons
(tendosynovitis) often leads to contractures of the fingers and Carpal Tunnel
Syndrome. (See related disorder section of this report for more information
on Carpal Tunnel Syndrome.) Trunk involvement occurs in approximately 50% of
reported cases, but the face is usually spared. Fatigue and weight loss are
common. Pain in the muscles (myalgia) and arthritis may also occur.
Skin changes are also common in the hands and feet, with the skin
appearing puckered and eventually losing its elasticity while developing a
consistency described as woody. Onset of skin changes is usually
characterized by a general feeling of ill health, low-grade fever and pains
in the joints and muscles. (See Related Disorder section for more
information on Carpal Tunnel Syndrome.)
Strenuous physical activity may intensify symptoms of Eosinophilic
Fasciitis. In rare cases, cardiac abnormalities, Sjogren's Syndrome,
Aplastic Anemia and Thrombocytopenia may develop. (For more information on
these disorders, choose "Sjogren," "Aplastic Anemia," and "Thrombocytopenia"
as your search terms in the Rare Disease Database.)
Causes
The exact cause of Eosinophilic Fasciitis is unknown. Some scientists
believe that it may be a variant of Scleroderma. Others believe it to be an
autoimmune disease. Autoimmune disorders occur when the body's natural
defenses (antibodies, lymphocytes, etc.), against invading organisms attack
perfectly healthy tissue.
In cases where onset of Eosinophilic Fasciitis has been related to
ingestion of the dietary supplement L-Tryptophan, patients have gotten
Eosinophilic Fasciitis after days, months or years of taking this amino acid.
In some cases of Eosinophilic Fasciitis, it is related to injection of L-
Tryptophan from one manufacturer. A trace contaminant in some lots of L-
tryptophan appears responsible.
An attack of Eosinophilic Fasciitis commonly follows strenuous physical
activity.
Scientists now believe they can confirm that L-tryptophan itself causes
symptoms of Eosinophilia Faciitis in patients who use the product. Further
studies have shown that L-tryptophan contaminated with 1-1-ethylidenebis
tryptophan (ETB) causes more severe problems in people than regular L-
tryptophan; still, L-tryptophan alone can cause Eosinophilia Faciitis.
Affected Population
Eosinophilic Fasciitis occurs most frequently in middle-aged men. It is also
more prevalent in Caucasians. In about 50% of reported cases there is a
history of unusual exertion or unaccustomed exercise preceding the onset of
the condition, and this exertion has occurred within a time span of a few
hours to several weeks of onset of Eosinophilic Fasciitis symptoms. The
Centers for Disease Control (CDC) is studying the cases of Eosinophilic
Fasciitis related to L-Tryptophan to determine why some people who have
ingested this dietary supplement have gotten Eosinophilic Fasciitis.
Related Disorders
Symptoms of the following disorder can be similar to those of Eosinophilic
Fasciitis. Comparisons may be useful for a differential diagnosis:
Scleroderma refers to a group of disorders characterized by degenerative
changes in the skin and by the chronic hardening and shrinking of the
connective tissues of any part of the body. In some cases, the disease may
progress to include the esophagus, intestines, kidneys, thyroid gland, heart
and lungs. Although the two disorders are very similar, there are several
important differences between Scleroderma and Eosinophilic Fasciitis. In
Eosinophilic Fasciitis, the fingers and toes are seldom involved; the skin is
often puckered rather than smooth and not devoid of hair as it is in
Scleroderma; the skin is often warm to the touch rather than cool as in
Scleroderma; and the involvement of other organs is not usually found in
Eosinophilic Fasciitis. (For more information on this disorder, choose
"Scleroderma" as your search term in the Rare Disease Database).
The following disorder may be associated with Eosinophilic Fasciitis as a
secondary characteristic. It is not necessary for a differential diagnosis:
Carpal Tunnel Syndrome is a common disorder caused by compression of the
peripheral nerves in one or both wrists. It is characterized by a sensation
of numbness, tingling, burning and/or pain in the hand and wrist. Persons
affected by this disorder may be awakened at night with the feeling that the
hand has "gone to sleep." Strain or injury involving the hand and wrist, or
various other disorders such as Eosinophilic Fasciitis may cause Carpal
Tunnel Syndrome. (For more information on this disorder, choose "Carpal
Tunnel" as your search term in the Rare Disease Database).
Therapies: Standard
Diagnosis of Eosinophilic Fasciitis is made by biopsy of the affected skin,
deep enough to include adjacent muscle fibers. Many individuals with
Eosinophilic Fasciitis respond favorably to corticosteroid therapy, and the
drug prednisone is commonly prescribed. Prednisone therapy may be required
for two months or longer. The drug cimetidine may be used as an alternative.
The most dramatic response to corticosteroids occurs early in the course of
the disease. Spontaneous and sometimes complete remission may occur
following an interval of two to five years. Sometimes, corticosteroid
therapy is not completely successful, and recurrence of the disorder has been
observed after discontinuation of therapy. Unfavorable responses to
corticosteroid therapy are usually related to the association of Eosinophilic
Fasciitis with Aplastic Anemia, Thrombocytopenia, Hemolytic Anemia and
Hodgkin's Disease. (For more information on these disorders, choose
"Aplastic Anemia," "Hemolytic Anemia," and "Hodgkin" as your search terms in
the Rare Disease Database.)
Therapies: Investigational
This disease entry is based upon medical information available through
January 1992. Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.
Resources
For more information on Eosinophilic Fasciitis, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
The National Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Information Clearinghouse
Box AMS
Bethesda, MD 20892
(301) 495-4484
Centers for Disease Control
1600 Clifton Road, NE
Atlanta, GA 30333
(404) 639-3534
Scleroderma Foundation
1725 York Avenue, Suite 29F
New York, NY 10123
(212) 427-7040
References
CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, M.D. et al., eds;
W.B. Saunders Company, 1988. Pp. 2021.
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, M.D., ed.-in-chief; Little,
Brown and Co., 1987. Pp. 1294-1296.
THE MERCK MANUAL, Volume 1, 14th Ed.: Robert Berkow, M.D., ed.-in-chief;
Merck Sharp & Dohme Laboratories, 1982. Pp. 1279-1280.
EOSINOPHILIC FASCIITIS IN A PAIR OF SIBLINGS. G.T. Thomson et al.;
ARTHRITIS RHEUM (January, 1989: issue 32 (1)). Pp. 96-99.
EOSINOPHILIC FASCIITIS: CLINICAL SPECTRUM AND THERAPEUTIC RESPONSE IN 52
CASES. S. Lakhanpal et al.; SEMIN ARTHRITIS RHEUM (May, 1988: issue 17 (4)).
Pp. 221-231.
Article on L-tryptophan use and Eosinophilia Myalgia featured in the
October 10, 1991 issue of NATURE. P. 490.