Hereditary hemorrhagic telangiectasia (HHT) is a rare genetic disease (ORPHA:774), with an estimated prevalence of approximately 1 in 5000 individuals, and is considered the second most common inherited bleeding disorder [1]. It is characterized by mucocutaneous telangiectasia and larger visceral vascular malformations (VMs), resulting in a broad and heterogeneous clinical spectrum that remains difficult to predict [2–4]. Clinical diagnosis is based on the Curaçao criteria (recurrent epistaxis, mucocutaneous telangiectasia, visceral involvement, and a HHT first-degree relative) [3,4].
