MANAGEMENT OF MULTIPLE ORAL LESIONS IN A RARE SYNDROME WITH PLATELETS IMPAIRMENT

Abstract

Hallerman-Streiff syndrome (HSS) is a rare congenital disease described in less than 200 cases worldwide. Skull changes, hypotrichosis, cataracts, a beaked nose, micrognathia, skin atrophy, dental anomalies, and short stature are characteristics described. A 28-year-old female patient was referred for dental care, complaining about recurrent spontaneous gingival bleeding. Intraoral examination revealed dental anomalies, periodontal disease, generalized gingival hyperplasia, and 3 nodules located on alveolar ridges. Due to extensive bleeding during periodontal treatment and medical history of bleeding episodes, a platelet aggregation test was requested, revealing impaired adrenaline-induced platelet aggregation. The following dental visits aimed to prevent hemorrhage during invasive procedures, using hemostatic agents. Nodules were removed with the aid of a high-power laser, with no intercurrences. Histologic analysis suggested the diagnosis of collagenous fibroma, a rare encounter. To date, this the first description of blood dyscrasia and oral lesions in a patients HSS.