Episodic angioedema with eosinophilia (Gleich syndrome) – a diagnostic challenge

Abstract

Episodic angioedema with eosinophilia, also known as Gleich syndrome, is an extremely rare disorder characterized by recurrent occurrence of angioedema, urticaria, fever, increased body weight, elevated serum values of immunoglobulin M, and leukocytosis with significant eosinophilia. The cause and pathogenesis of the disease are not fully clarified. It occurs mostly in children or adults of younger age. The first choice of treatment are glucocorticoids, and the prognosis is mostly good. We report a case of a patient who was diagnosed with Gleich syndrome based on recurrent episodes of angioedema and fever in the presence of leukocytosis with eosinophilia and elevated immunoglobulin M values. The patient has been in stabile remission for four years since the administration of glucocorticoids.

Keywords

• angioedema – complications
• diagnosis