Episodic angioedema with eosinophilia

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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 .

Epidemiology

Episodic angioedema with eosinophilia is rare, only about 50 cases have been published in the literature to date.

Clinical presentation

Typically an episodic condition with new attacks every month or so, which self-resolve without any external interventions being needed.

  • urticaria
  • fever
  • angioedema
  • 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 .