Wells syndrome

Title

Other Names:

Eosinophilic cellulitis; Bullous cellulitis with eosinophilia; Wells' syndrome

Categories:

Skin Diseases

Summary Summary

Wells syndrome is a rare eosinophilic disorder that primarily affects the skin. Affected people typically develop a skin rash that is often proceeded by itching or burning skin. The rash consists of raised, red, swollen areas that may be warm to the touch. The symptoms generally come on rapidly and may last four to eight weeks. In some cases, the rash may recur (occur frequently or repeatedly) for years. The exact underlying cause of Wells syndrome is currently unknown; however, some scientists believe that it may be an autoimmune reaction. Oral or topical corticosteroids are commonly used to treat Wells syndrome, although antifungal drugs, antibiotics, immunosuppressants, and/or antihistamines have also been used with varying degrees of success.^[1]^[2]^[3]

Last updated: 4/27/2015

Symptoms Symptoms

People affected by Wells syndrome generally develop a skin rash that is often proceeded by itching or burning skin. The rash consists of raised, red, swollen areas that may be warm to the touch. The limbs (arms and legs) are the most commonly affected area of the body; however, the trunk may be involved, as well. The symptoms generally come on rapidly and may last four to eight weeks. In some cases, the rash may recur (occur frequently or repeatedly) for years.^[1]^[2]^[3]

Some affected people may experience other symptoms including asthma, joint pain, fever, and fatigue.^[2]^[3]

Last updated: 4/27/2015

The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.

Signs and Symptoms	Approximate number of patients (when available)
Abnormality of eosinophils	90%
Cellulitis	90%
Edema	90%
Pruritus	90%
Skin rash	90%
Abnormal blistering of the skin	50%
Urticaria	50%

Last updated: 9/1/2016

Cause Cause

The exact underlying cause of Wells syndrome is currently unknown. However, some scientists believe that it may be an autoimmune reaction. In people affected by an autoimmune disorder, the body's immune system mistakenly attacks it's own healthy tissues.^[1]

Possible triggers for Wells syndrome include:^[3]

Bug bites (i.e. spiders, bees, fleas, ticks, or mites)
Viral infections
Parasitic infections
Leukemia
Myeloproliferative disorders
Atopic dermatitis
Fungal infections
Certain types of medications
Churg-Strauss syndrome

Last updated: 4/27/2015

Diagnosis Diagnosis

A diagnosis of Wells syndrome is often suspected based on the presence of characteristic signs and symptoms. A skin biopsy may then be recommended to support the diagnosis.^[2]^[3] It is also important to exclude possible triggers of the condition which may require unique treatment protocols.^[2]

Last updated: 4/27/2015

Treatment Treatment

The skin symptoms associated with wells syndrome are typically treated with oral or topical corticosteroids such as Prednisone. Other medications that may be used to treat this condition include antifungal drugs, antibiotics, immunosuppressants, and/or antihistamines (H1 receptor antagonists).^[2]^[3]

Medscape Reference's Web site offers more specific information about the different medications that are often used to treat Wells syndrome. Please click the link to access this resource.

Last updated: 4/27/2015

Prognosis Prognosis

The long-term outlook (prognosis) for people with Wells syndrome is excellent. The condition usually resolves over the course of four to eight weeks and may clear up more quickly with treatment. Most people do not have residual scarring.^[2]^[3]

In some affected people, the rash may recur (occur frequently or repeatedly). These cases may take years to ultimately resolve.^[3]

Last updated: 4/27/2015

Do you have updated information on this condition? Let us know.

Organizations Organizations

Nonprofit support and advocacy groups bring together patients, families, medical professionals, and researchers. These groups often raise awareness, provide support, and develop patient-centered information. Many are the driving force behind research for better treatments and possible cures. They can direct people to research, resources, and services. Many groups also have experts who serve as medical advisors. Visit their website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue Eastpointe
East Detroit, MI 48201-2227
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: http://www.aarda.org/
American Partnership For Eosinophilic Disorders
PO Box 29545
Atlanta, GA 30359
Telephone: 713-493-7749
E-mail: mail@apfed.org
Website: http://www.apfed.org
International Eosinophil Society
555 East Wells Street, Suite 1100
Milwaukee, WI 53202
Telephone: 414-276-3349
E-mail: kfilipiak@eosinophil-society.org/
Website: http://www.eosinophil-society.org/

Do you know of an organization? Send us your suggestions.

Living With Living With

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Healthcare Resources

Cincinnati Center for Eosinophilic Disorders
Toll-free: 800-344-2462
Telephone: 513-636-2233
E-mail: cced@cchmc.org
Website: http://www.cincinnatichildrens.org/service/c/eosinophilic-disorders/default/

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Wells syndrome. Click on the link to view a sample search on this topic.

News & Events News & Events

NCATS Co-Sponsored Conferences

8th International Eosinophil Society’s Biennial Symposium Saturday, July 13, 2013 - Wednesday, July 17, 2013
Location: Oxford, England
Description: IES Symposium organizers’ central goal is to provide a highly interactive, interdisciplinary forum for scientific exchange and collaboration amongst junior and senior scientists in the fields of allergy, immunology, hematology, and cancer in relation to the role of the eosinophil in health and disease.

Contact: Michael Minnicozzi, Ph.D., (301) 451-0140minnicozzim@niaid.nih.gov

Co-funding Institute(s): National Institute of Allergy and Infectious Diseases, Office of Rare Diseases Research

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

My child has been having outbreaks from Wells since infancy, but nobody knew what was going on. It took a number of years before he was finally diagnosed with Wells syndrome. For the last two years he has taken a low dose steroid every other day. When his outbreaks are really bad his doctors just give him more steroids. The steroids help it not get out of hand like it was before he started the medicine, however I worry about the long term effects. We've tried Griseofulvin in the past, but that did not help at all. Are there other treatment options? See answer
I have been diagnosed with Wells syndrome. How is this condition treated? See answer

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