Osler-Weber-Rendu syndrome--dental implications.
Osler-Weber-Rendu syndrome (OWRS) is a rare hereditary, autosomal dominant disease characterized by a local angiodysplasia. Its clinical characteristics are vascular hamartomas of the skin and oral mucosa, arteriovenous malformations in the lungs, liver, kidney and brain, and episodes of epistaxis. The oral lesions, which become apparent through hemorrhagic telangiectasia, may be the first sign of the disease. This is a case report of a 74-year-old woman whose diagnosis of OWRS was established by her dentist based on the presence of telangiectasia in the skin and oral mucosa, reports of frequent nosebleeds of unknown etiology and a family history of telangiectasia. Amputation of a lower limb and comorbidities, such as cardiopathy, nephropathy and rheumatic disorders, completed the profile. OWRS causes major vascular changes that can be diagnosed initially by a dentist. In this article, we describe the skills and knowledge that dentists need to monitor patients with OWRS properly.
da Silva Santos
Paulo Sérgio
PS
Department of Oral Pathology, School of Dentistry, University of São Paulo, São Paulo, Brazil.
Fernandes
Karin Sá
KS
Magalhães
Marina Helena
MH
D000368:Aged; D017742:Dental Care for Chronically Ill; D005260:Female; D006801:Humans; D009059:Mouth Diseases / Q000209:etiology*; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000175:diagnosis*