Revista Portuguesa de Medicina Geral e Familiar (Apr 2024)

Hereditary angioedema with normal C1-inhibitor levels: a rare case report

  • Filipa Rodrigues dos Santos,
  • Inês Falcão,
  • Leonor Cunha

DOI
https://doi.org/10.32385/rpmgf.v40i2.13745
Journal volume & issue
Vol. 40, no. 2

Abstract

Read online

Hereditary angioedema (HAE) is a rare, hereditary disease and its manifestations may be life-threatening. It differs from histaminergic angioedema since it shows different underlying mechanisms and, therefore, does not respond to antihistamine or adrenaline treatment. The authors present a case of hereditary angioedema to highlight the importance of prompt referral to the allergy and clinical immunology for further evaluation and correct diagnosis in case of suspicion of this entity.

Keywords