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Disease Profile
Episodic angioedema with eosinophilia
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.
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Age of onset
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ICD-10
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Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
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.
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.
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.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
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.
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.
Not applicable
Other names (AKA)
Gleich syndrome
Summary
Episodic angioedema with eosinophilia is a disorder characterized by episodes of swelling under the skin (angioedema) and an elevated number of the
Symptoms
- Swelling under the skin (angioedema)
- Hives (urticaria)
- Fever
- Swelling
- Weight gain
- Reduced production of urine
Laboratory findings during these episodes may include:[2][4]
- An elevated number of the
white blood cells known as eosinophils (eosinophilia) - Elevated levels of certain
antibodies , including IgG and IgM
The episodes usually occur every 3-4 weeks and resolve on their own within several days. There may be an increase in urine production once symptoms resolve.[3]
Cause
Treatment
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
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American Partnership For Eosinophilic Disorders
PO Box 29545
Atlanta, GA 30359
Telephone: 713-493-7749
E-mail: mail@apfed.org
Website: https://www.apfed.org -
International Eosinophil Society
555 East Wells Street, Suite 1100
Milwaukee, WI 53202
Telephone: 414-276-6445
E-mail: info@eosinophil-society.org/
Website: https://www.eosinophil-society.org/
References
- O'Hollaren MT. The Hypereosinophilic Syndromes. Medscape Reference. 2006; https://www.medscape.org/viewarticle/520117.
- Khoury P, Herold J, Alpaugh A, Dinerman E, Holland-Thomas N, Stoddard J et al. Episodic angioedema with eosinophilia (Gleich syndrome) is a multilineage cell cycling disorder. Hematologica. March, 2015; 100(3):300–307. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4349267/.
- Roufosse F, Klion AD & Weller PF. Hypereosinophilic syndromes: Clinical manifestations, pathophysiology, and diagnosis. UpToDate. August 17, 2017; https://www.uptodate.com/contents/hypereosinophilic-syndromes-clinical-manifestations-pathophysiology-and-diagnosis.
- Liu F, Hu W, Liu H, Zhang M, Sang H. Episodic angioedema associated with eosinophilia. Anais Brasileiros de Dermatologia. Jul-Aug, 2017; v.92(4):https://www.ncbi.nlm.nih.gov/pubmed/28954105.
- Wright BL, Butterfield JH, Leiferman KM, Gleich GJ. Development of Eosinophilic Endomyocardial Disease in a Patient with Episodic Angioedema and Eosinophilia. The journal of allergy and clinical immunology In practice. 2016; 4(2):336-337. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4955938/.
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