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Gleich syndrome: a systematic review.

Roger Haber1, Joanna A Chebl2, Maria El Gemayel3

  • 1Dermatology Department, Faculty of Medicine, Balamand University, Saint Georges Hospital UMC, Beirut, Lebanon.

International Journal of Dermatology
|June 20, 2020
PubMed
Summary

Gleich syndrome, a rare disorder causing hypereosinophilia and angioedema, presents with symptoms like fever and urticaria. Systemic corticosteroids are effective treatments, though further research into its pathophysiology is needed.

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Area of Science:

  • Rare autoimmune disorders
  • Clinical immunology
  • Dermatology

Background:

  • Gleich syndrome is a rare disorder characterized by hypereosinophilia and angioedema.
  • Clinical and therapeutic findings remain unclear, with symptoms including elevated IgM, weight gain, fever, and urticaria.

Purpose of the Study:

  • To review Gleich syndrome, assessing its clinical features, epidemiology, and treatment options.
  • To consolidate current knowledge on this rare hypereosinophilic entity.

Main Methods:

  • A systematic review of 32 articles, including case reports and series, focusing on eosinophilic angioedema and Gleich syndrome.
  • Data extraction included patient demographics, clinical and biological manifestations, and treatment protocols.

Main Results:

  • Common clinical findings include recurrent angioedema, fever, urticaria, weight gain, blood eosinophilia, and elevated immunoglobulin levels.
  • Corticosteroid therapy is identified as the primary treatment modality.

Conclusions:

  • Gleich syndrome is a distinct hypereosinophilic condition with a generally benign course.
  • The syndrome shows a favorable response to systemic corticosteroids, but further research is required to elucidate its pathophysiology and optimize therapeutic strategies.