Dental manifestations of hereditary hemorrhagic telangiectasia: a clinical case

Abstract

A rare disorder that changes the blood vessels of many organs and causes bleeding. The disease affects the lungs, liver, central nervous system, gastrointestinal tract, skin and mucous membranes. The degree of incidence of Randu-Weber-Osler disease is not high and ranges from 1 detected case per 50 thousand – per 100 thousand population. The scientific article presents a clinical case with dental manifestations of Randu-Weber-Osler disease and indicates the important role of the dentist in monitoring such patients. The red border of the lips is dry, pale, with pronounced multiple punctate telangiectasias of red-violet color. In the area of the lower lip, brightly colored round nodules with a diameter of about 5 mm are found, protruding above the surface, painless on palpation. On the dorsal and lateral surfaces, there are papular formations of a red-violet color, with a diameter of 5 to 7 mm, protruding above the surface. The gums are hyperemic, edematous, loose, bleeds on probing, periodontal pockets with exudative contents. On examination, attention was drawn to an insufficient amount of saliva, which was located mainly in the retromolar region; it was frothy. The salivation rate is reduced (0.1 ml per minute). It is assumed that before and during invasive dental procedures, antibiotic therapy is necessary, especially when working in infected areas (500 mg of amoxicillin) every 8 hours starting 12 hours before the procedure for 7 days. Given that hypoxia and cerebral embolism are frequent complications, the dental chair should be in an upright position throughout the patient's appointment. Treatment of this disorder is only supportive in nature, its goals are to relieve symptoms, treat complications and prevent progression of the disease. Early definition of the manifestations of this disease and careful observation of the patient gives a favorable prognosis of the course and prevents the development of severe complications.

Keywords: hereditary hemorrhagic telangiectasia, periodontitis, xerostomia.