A rare condition affecting the primary and permanent dentition: Dentin dysplasia type I

Dentin dysplasia type I is a genetic defect of dentin formation, which is inherited as an autosomal dominant trait. In this anomaly, the teeth generally appear unremarkable with normal crowns; and it is characterized by normal appearing crowns, absence or severe restriction of root formation, obliterated pulp chambers, and periapical radiolucencies without an obvious cause. Clinically, the teeth can be poorly aligned, drifting and mobile, and can prematurely exfoliate due to abnormal root formation. This condition is rarely encountered in dental practice. Little is known about the specific treatment of this disorder and management of patients with dentin dysplasia has presented with problems. This report documents a rare case of DD type I in a 7-year-old girl and the clinical, radiographical and histopathological findings of this condition.