Hereditary angio-oedema: Long-term prophylaxis with danazol

Abstract

Hereditary angio-oedema is a rare autosomal dominant disease characterized by recurrent attacks of subcutaneous oedema and caused by a deficiency of the plasma protein C1 inhibitor. Hereditary angio-oedema attacks carry a high risk of morbidity or even mortality. In literature, there are only a few publications on the treatment and follow-up of paediatric patients. We report the case of a 14-year-old male with known hereditary angio-oedema since the age of six years, revealed by a recurrent acute abdominal pain and oedema in subcutaneous tissues of extremities and face, and he was admitted once for sub mucosal oedema of larynx with suffocation, treated by fresh-frozen plasma with favourable outcome. Danazol was used for long-term prophylaxis. Clinical course improved over a follow-up of 12 months.