Intra and extra oral clinical manifestations of Rendu-Osler-Weber syndrome: case report and literature review

Abstract

Hereditary Hemorrhagic Telangiectasia or Rendu-Osler-Weber syndrome is an incomplete penetrance dominant autosomal transmission disease which determines microcirculatory beds alterations (capillary and venules), caused by the loss of the support tissues that usually enclose blood vessels, and hemorrhage potentially in every organ. The syndrome clinical manifestations are multiple telangiectasia of small proportions on the skin or on the mucous membranes (e.g. of the gastrointestinal tract or other organs), in association with recurring bleedings of the affected areas and external and internal melena. The treatment is a supportive one so to prevent complications. This study reports a case of a patient affected by this syndrome in need of a dental implant following the fracture of a tooth. Furthermore, a bibliographical review of etiopathogenesis, clinical manifestations and therapy options has been made.