Episodic angioedema with eosinophilia
Episodic angioedema with eosinophilia (EAE), also known as Gleich syndrome, is a rare condition presenting as a self-limiting cyclic urticaria, fever, angioedema, weight gain and marked eosinophilia, with 3-4 weekly episodes 1.
Episodic angioedema with eosinophilia is rare, only about 50 cases have been published in the literature to date.
Typically an episodic condition with new attacks every month or so, which self-resolve without any external interventions being needed.
- weight gain (~15-20% body weight)
- marked eosinophilia
Treatment and prognosis
It generally spontaneously resolves, although when presentation is severe, steroids are sometimes administered to quicken recovery.
Its natural history is benign with no known involvement of the internal viscera.
History and etymology
The first four cases were reported by an American immunologist Gerald J Gleich and colleagues in 1984 2.
- 1. Khoury P, Herold J, Alpaugh A, Dinerman E, Holland-Thomas N, Stoddard J, Gurprasad S, Maric I, Simakova O, Schwartz LB, Fong J, Lee CC, Xi L, Wang Z, Raffeld M, Klion AD. Episodic angioedema with eosinophilia (Gleich syndrome) is a multilineage cell cycling disorder. (2015) Haematologica. 100 (3): 300-7. doi:10.3324/haematol.2013.091264 - Pubmed
- 2. Gleich GJ, Schroeter AL, Marcoux JP, Sachs MI, O'Connell EJ, Kohler PF. Episodic angioedema associated with eosinophilia. (1984) The New England journal of medicine. 310 (25): 1621-6. doi:10.1056/NEJM198406213102501 - Pubmed
- 3. Liu F, Hu W, Liu H, Zhang M, Sang H. Episodic angioedema associated with eosinophilia. (2017) Anais brasileiros de dermatologia. 92 (4): 534-536. doi:10.1590/abd1806-4841.20174351 - Pubmed