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Familial Eosinophilic Cellulitis

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

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Synonyms of Familial Eosinophilic Cellulitis

Disorder Subdivisions

General Discussion

Familial eosinophilic cellulitis is a rare skin disorder. It is characterized by raised, red, swollen, and warm areas of skin, in a flame-shaped pattern with associated pain. The exact cause of the disease is unknown. However, bites of spiders, bees, mites, fleas, or ticks (arthropods) are often associated with this skin condition.

Symptoms

Familial eosinophilic cellulitis is a rare skin disorder. It sometimes occurs as an exaggerated response to bites of spiders, bees, fleas, ticks, or mites (arthropods), or it may have other causes such as surgery or drugs. The skin of the person will develop flame shaped patterns of raised, swollen, red areas that are warm to the touch. The episodes usually come on rapidly. Often, familial eosinophilic cellulitis will recur suddenly over a period of years with swelling and redness developing for no apparent reason. The attack may last up to six weeks and may continue to recur for years.

Large areas of skin may be affected and testing shows microscopic changes of the tissue. An abnormal number of white blood cells (eosinophils) are found in the red and swollen areas of skin, underlying fat, and usually in the blood. Skin blistering has also been known to develop.

Causes

The exact cause of familial eosinophilic cellulitis is still not known. Some scientists believe that there may be an autoimmune basis for 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.

Affected Populations

Familial eosinophilic cellulitis affects males and females in equal numbers. The disorder is more often found in adults, but it may strike children as well.

Related Disorders

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

Cellulitis is characterized by inflamed tissue of the skin. Often the skin becomes red, swollen, and painful, over a large area. There may be accompanying chills and fever. This disorder can be caused by either Group A beta-hemolytic streptococci, or in older persons it is sometimes caused by Group G streptococci.

Anaphylaxis is an extreme allergic reaction that can be caused by a person's hypersensitivity to drugs, insect venom, fish, nuts, and other substances. There is often extreme swelling, itching, flushing, hives, and other physical reactions to a particular substance. These reactions can often be life-threatening. (For more information on this disorder, choose "Anaphylaxis" as your search term in the Rare Disease Database.)

Contact dermatitis is a common allergic disorder characterized by skin inflammation and blisters. Redness, swelling, oozing, crusting, scaling, burning pain and usually itching are common. (For more information on this disorder choose "Contact Dermatitis" as your search term in the Rare Disease Database.)

Standard Therapies

Standard treatment of familial eosinophilic cellulitis may consist of administration of steroid drugs. However, the disorder often resolves itself after a number of weeks. Other treatment is symptomatic and supportive.

Investigational Therapies

Research on Autoimmune diseases is continuing to determine why these disorders occur and how to treat them. For more information about this research contact the agencies listed in the Resources section of this report.

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

Organizations related to Familial Eosinophilic Cellulitis

References

TEXTBOOKS
Davis MDP, Leiferman KM. Familial Eosinophilic Cellulitis (NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:99.

Champion RH, Burton JL, Ebling FJG. Eds. Textbook of Dermatology. 5th ed. Blackwell Scientific Publications. London, UK; 1992.

Habif TP. Ed. Clinical Dermatology. 2nd ed. The C.V. Mosby Company. St. Louis, MO; 1990.

REVIEW ARTICLES
Holme SA, McHenry P. Nodular presentation of eosinophilic cellulitis (Wells' syndrome). Clin Exp Dermatol. 2001;26:677-79.

Weiss G, Shemer A, Confino Y, et al. Wells' syndrome: report of a case and review of the literature. Int J Dermatol. 2001;40:148-52.

Delaporte E. [From Wells syndrome to "eosinophilic disease"] Ann Dermatol Venereol. 2001;128
(3 Pt 1):207-11.

JOURNAL ARTICLES
Moossavi M, Mehregan DR. Wells' syndrome: a clinical and histopathologic review of seven cases. Int J Dermatol. 2003;42:62-67.

Tsuji Y, Kawashima T, Yokota K, et al. Wells' syndrome as a manifestation of hypereosinophilic syndrome. Br J Dermatol. 2002;147:811-12.

Herr H, Koh JK. Eosinophilic cellulitis (Wells' syndrome) successfully treated with low-dose cyclosporine. J Korean Med Sci. 2001;16:664-68.

Seckin D, Demirhan B. Drugs and Wells' syndrome: a possible causal relationship? Int J dermatol. 2001;40:138-40.

FROM THE INTERNET
Brown J, Schwartz RA. Wells Syndrome (Eosinophilic Cellulitis). eMedicine. Last Updated: August 20, 2002:12 pp.
www.emedicine.com/derm/topic908.htm

Report last updated: 2009/05/14 00:00:00 GMT+0