National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Episodic angioedema with eosinophilia


Other Names:
Gleich syndrome
This disease is grouped under:
Episodic angioedema with eosinophilia is a disorder characterized by episodes of swelling under the skin (angioedema) and an elevated number of the white blood cells known as eosinophils (eosinophilia). During these episodes, symptoms of hives (urticaria), fever, swelling, weight gain and eosinophilia may occur. Symptoms usually appear every 3-4 weeks and resolve on their own within several days. Other cells may be elevated during the episodes, such as neutrophils and lymphocytes.[1][2] Although the syndrome is often considered a subtype of the idiopathic hypereosinophilic syndromes, it does not typically have organ involvement or lead to other health concerns.[1][3] The cause of this condition is unknown. Treatment may not be needed, but can involve the use of steroids.[2] 
Last updated: 4/12/2018
People with episodic angioedema associated with eosinophilia (EAE) experience recurring episodes of the following symptoms:[2][3][4]
  • 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]
Last updated: 4/12/2018
The cause of episodic angioedema with eosinophilia (EAE) is not well understood. Experts suspect that certain proteins in the immune system called interleukins may play a role. Specifically, interleukin 5 (IL-5) levels are elevated during episodes. Studies have lead to the belief that these proteins play a role in the inflammatory process involved in EAE.[4]
Last updated: 4/12/2018
Treatment may not be needed for episodic angioedema with eosinophilia (EAE), since symptoms usually resolve on their own. When treatment is initiated, steroids are usually the first choice. Certain medications used to treat cancer, including Imatinib (Gleevac) have also been successfully used in treating EAE.[4]
Last updated: 4/12/2018
Generally, episodic angioedema with eosinophilia (EAE) is considered to be a benign condition with a self limiting course. Typically, other organs are not involved and over time, episodes may resolve on their own.[12728] In some instances, people with EAE can develop symptoms in other organs, such as cardiac disease.[5] Symptoms may also progress to other types of non episodic hypereosinophilic syndrome.[3]
Last updated: 4/12/2018

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  1. O'Hollaren MT. The Hypereosinophilic Syndromes. Medscape Reference. 2006; https://www.medscape.org/viewarticle/520117.
  2. 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/.
  3. Roufosse F, Klion AD & Weller PF. Hypereosinophilic syndromes: Clinical manifestations, pathophysiology, and diagnosis. UpToDate. August 17, 2017; http://www.uptodate.com/contents/hypereosinophilic-syndromes-clinical-manifestations-pathophysiology-and-diagnosis.
  4. 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.
  5. 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/.