Episodic Angioedema with Eosinophilia (EAE), also known as Gleich’s Syndrome is a rare eosinophilic disorder characterized by recurrent episodes of fever, swelling (described as angioedema) that is sometimes associated with hives (urticaria). Some patients can experience significant weight gain, lymph node swelling, muscle pain, fatigue or rashes that occur in 3-6 week intervals. Symptoms spontaneous resolve often in the absence of therapy. Laboratory studies often show elevated neutrophils and eosinophils at the onset of symptoms that last over a week with improvement of the counts after two weeks. In some individuals the eosinophil count doesn’t return to the normal range between the cycles.
Other laboratory findings that are seen in EAE are an elevated IgM, and presence of T cells with a characteristic phenotype (CD3-CD4+). Eosinophils or their products are often observed in the skin when biopsies are taken, and imaging studies may show inflammation in the muscles of areas that are affected. Some patients are unable to tolerate the recurrent symptoms associated with pain, recurrent weight changes, and the impact on their quality of life and require treatment with glucocorticoids. No other medications have been formally studied in this rare disease.
As with all hypereosinophilic syndromes, monitoring for development of other organ involvement or progression of disease is important.
Episodic angioedema associated with eosinophilia. Gleich GJ, Schroeter AL, Marcoux JP, Sachs MI, O’Connell EJ, Kohler PF. Trans Assoc Am Physicians. 1984;97:25-32.
Episodic angioedema associated with eosinophilia. Gleich GJ, Schroeter AL, Marcoux JP, Sachs MI, O’Connell EJ, Kohler PF. N Engl J Med. 1984 Jun 21;310(25):1621-6.
Episodic angioedema with eosinophilia (Gleich syndrome) Schiavino D, Gentiloni N, Murzilli F, Gebreselassie M, La Rocca LM, Patriarca G. Allergol Immunopathol (Madr). 1990 Jul-Aug;18(4):233-6. Review.
Contributing Authors and Reviewers
Paneez Khoury, MD
Gerald Gleich, MD.