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Disease Profile

Wells syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
<1 / 1 000 000

< 331

US Estimated

< 514

Europe Estimated

Age of onset

Adult

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ICD-10

L98.3

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Eosinophilic cellulitis; Bullous cellulitis with eosinophilia; Wells' syndrome

Categories

Skin Diseases

Summary

Wells syndrome is a rare eosinophilic disorder that primarily affects the skin. Affected people typically develop a skin rash that is often preceded 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 reactionOral 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]

Symptoms

People with Wells syndrome generally develop a skin rash that is often preceded 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. Symptoms generally come on rapidly and may last for four to eight weeks. In some cases, the rash may recur (occur frequently or repeatedly) for years.[1][2][3] Some people with Wells syndrome may experience symptoms that do not affect the skin, such as asthma, joint pain, fever, or fatigue.[2][3]

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Cellulitis
Bacterial infection of skin
Skin infection

[ more ]

0100658
Pruritus
Itching
Itchy skin
Skin itching

[ more ]

0000989
30%-79% of people have these symptoms
Abnormal blistering of the skin
Blistering, generalized
Blisters

[ more ]

0008066
Edema
Fluid retention
Water retention

[ more ]

0000969
Eosinophilia
High blood eosinophil count
0001880
Skin vesicle
0200037
5%-29% of people have these symptoms
Arthralgia
Joint pain
0002829
Fever
0001945

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]

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]

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.

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s 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

    Organizations Providing General Support

      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.

          References

          1. Familial eosinophilic cellulitis. National Organization for Rare Disorders (NORD). 2009; https://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1003/viewAbstract. Accessed 12/14/2011.
          2. Wells syndrome. DermNet NZ. December 2013; https://dermnetnz.org/reactions/wells.html.
          3. Robert A Schwartz, MD, MPH. Wells Syndrome. Medscape Reference. May 2014; https://emedicine.medscape.com/article/1124844-overview.
          4. Schwartz RA. Wells Syndrome (Eosinophilic Cellulitis). eMedicine. May 2011; https://emedicine.medscape.com/article/1124844-clinical#a0218. Accessed 12/14/2011.

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