Hereditary hemorrhagic telangiectasias: a challenge for the clinician

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) remains a challenge for all clinicians, as in about 80% of cases nasal bleeding is t he first manifestation of this disease, which is characterized by a clinica l triad of multiple telangiectasias, recurrent hemorrhages and familial occ urrence. Although in the last few years there has been diagnostic and thera peutic progress, a cure for this rarely life-threatening but often burdenin g and handicapping disease is still not possible. We have reviewed head and neck presentations, diagnostic and therapeutic features, as well as new in sights into the molecular genetics of the disease and local treatment now a vailable.