The differences with HAE due to C1inh deficiency rely on the sensitivity to estrogens of these forms [2, 12], especially those related to F12 pathogenic variants: almost all symptomatic patients are women, and female hormonal events (pregnancy, contraception, menstruations, etc.)are more frequently a trigger for angioedema attacks [2–5]. This evidence concerns the gene SERPING1 and hereditary angioedema.