OROFACIAL FEATURES AND MEDICAL PROFILE IN A PATIENT WITH TUBEROUS SCLEROSIS COMPLEX

Tuberous sclerosis complex (TSC) is a rare autosomal-dominant multisystemic disorder caused by mutation in the TSC1/TSC2 genes. TSC is characterized by cognitive impairment, seizures, and the presence of benign tumors in multiple organs, including oral mucosa. Among oral findings, enamel hypoplasia, oral fibromas, cleft palate, and alterations of maxillary bone trabeculation are the most frequent. The present report describes the case of a melanodermic female, 41 years old, showing dermatologic and neurologic signs of TSC, with a medical history of epilepsy, cognitive deficit, chronic renal failure, arterial hypertension, cardiac arrhythmia, anemia, and hypothyroidism. Intra-oral exam showed multiple papular lesions in the bilateral buccal mucosa with a diagnosis of inflammatory fibrous hyperplasia and red macule on the hard palate, not associated with prothesis use and without change during the period of treatment. The exfoliative cytologic test was negative for candidiasis, and dental treatment was performed to promote a better quality of life for the patient.