Published: 7 September 2023
Author(s): Pau Cerdà, Sandra D. Castillo, Cinthia Aguilera, Adriana Iriarte, José Luis Rocamora, Ane M. Larrinaga, Francesc Viñals, Mariona Graupera, Antoni Riera-Mestre
Section: Original article

Hereditary hemorrhagic telangiectasia (HHT) or Rendu–Osler–Weber syndrome (ORPHA:774) is a rare autosomal dominant vascular disease characterized by the presence of telangiectases and larger vascular malformations (VM), mainly in pulmonary, hepatic or cerebral vasculature [1]. Telangiectasis is the main characteristic lesion in HHT and consists in dilated postcapillary venules directly connected to dilated arterioles bypassing the normal capillary bed [2]. Nasal or gastrointestinal mucosae telangiectases are more prone to hemorrhage, causing epistaxis, gastrointestinal bleeding and anemia, being these the clinical hallmarks of the disease [3].

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