Hereditary Angioedema in Female of Three Generations

Date of submission: 14-10-2017 | Date of acceptance: 26-04-2018 | Published: 06-11-2018

  • Rita Magalhães Moita Paediatrics Department, Hospital Pediátrico Integrado, Centro Hospitalar São João, Oporto, Portugal
  • Sylvia Jacob Paediatrics Department, Hospital Pediátrico Integrado, Centro Hospitalar São João, Oporto, Portugal
  • Cristina Ferreras Paediatrics Department, Hospital Pediátrico Integrado, Centro Hospitalar São João, Oporto, Portugal
  • Artur Bonito Vítor Paediatrics Department, Hospital Pediátrico Integrado, Centro Hospitalar São João, Oporto, Portugal | Allergology and Primary Immunodeficiencies Unit, Hospital Pediátrico Integrado, Centro Hospitalar São João, Oporto, Portugal

Abstract

Hereditary angioedema type III is a type of angioedema, affecting mainly females, without alteration of the level or function of C1 inhibitor, and is associated with exposure to increased levels of oestrogens. The authors present the case of a previously healthy 14-year-old girl observed with lip and face oedema with four days of evolution.
She had started an oral contraceptive about one week before. She had no new episodes of angioedema after stopping the oral contraceptive. In the family history, both the grandmother and great-grandmother had episodes of recurrent angioedema until menopause.
The authors intend to discuss a rare form of angioedema with an important impact on the patient’s quality of life.
The early diagnosis is important and should be based on clinical findings and a detailed family history.

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Published
2018-11-06
Section
Case reports