Familial Eosinophilic Cellulitis

Familial Eosinophilic Cellulitis

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Familial Eosinophilic Cellulitis 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

  • Eosinophilic Cellulitis
  • Granulomatous Dermatitis with Eosinophilia
  • Wells syndrome

Disorder Subdivisions

  • None

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.

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

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

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

1600 Clifton Road NE

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

6610 Rockledge Drive, MSC 6612

Bethesda, MD 20892-6612

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/



American Academy of Allergy, Asthma and Immunology

611 East Wells Street

Milwaukee, WI 53202

Tel: (414)272-6071

Fax: (414)276-3349

Tel: (800)822-2762

Email: info@aaaai.org

Internet: http://www.aaaai.org



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



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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