Intra- and extra-oral clinical manifestations of the Rendu-Osler-Weber syndrome

OBJECTIVES. Hereditary hemorrhagic telangectasia, also known as the Rendu-Osler-Weber syndrome, is a rare autosomal dominant form of fibrovascular dysplasia with incomplete penetrance. It is characterized by structural alterations at the microcirculatory level (capillaries and venules) related to the loss of supportive tissues and potential hemorrhagic involvement of all organs. MATERIALS AND METHODS. This report provides a review of the literature on the pathogenesis, clinical manifestations, and treatment options of Rendu-Osler-Weber syndrome and describes a case recently treated in the Oral Medicine ward of the University of Milan (Bicocca). RESULTS AND CONCLUSIONS. The syndrome is manifested by multiple small telangectasias involving the skin and mucosa (e.g., that of the gastrointestinal tract or other organs) associated with recurrent episodes of bleeding at involved sites and frank or occult melena. Treatment is supportive and aimed at the prevention of complications. In patients diagnosed with hereditary telangectasia, broad spectrum antibiotic prophylaxis is essential before all procedures, particularly those that are invasive (e.g., extractions, implants, etc.). The authors suggest the use of amoxicillin 1 g every 12 h, starting the day before the procedure. Treatment should be continued for 5 more days.