Gopalakrishnan Sivakumar, Balasubramaniam Nandakumar, Ramamoorthi Raghini, Vedachalam Rajarajeswari
Department of Oral Pathology, Madha Dental College and Hospital, Chennai, Tamil Nadu, India.
Department of Orthodontics, Meenakshi Ammal Dental College, Chennai, Tamil Nadu, India.
J Oral Maxillofac Pathol. 2021 Sep-Dec;25(3):559. doi: 10.4103/jomfp.jomfp_337_20. Epub 2022 Jan 11.
Dentin dysplasia is a rare, hereditary disorder affecting the dental hard tissue. It is a congenital, autosomal dominant disease of unknown etiology that affects 1:100,000 populations. It may present as such affecting only the dental hard tissue or as one of the symptoms of underlying diseases such as calcinosis, Ehlers-Danlos syndrome, rhematoid arthitis, Vitaminosis D and Branchioskeletogenital syndrome. This was first described by Ballschmiede as rootless teeth in 1920 and termed as dentin dysplasia by Rushton in the year 1939. It is classified into Type I, II and III, in which Type III affects only the secondary dentition. This article reports a rare case of Type I dentin dysplasia in a 26-year-old male patient, and focus on clinical, radiological, ground section and histopathological aspects. It emphasizes the significance of early diagnosis and intervention for the psychological well-being of the individual.
牙本质发育异常是一种罕见的遗传性疾病，影响牙齿硬组织。它是一种病因不明的先天性常染色体显性疾病，发病率为1/100000。它可能仅表现为影响牙齿硬组织，也可能是诸如钙质沉着、埃勒斯-当洛综合征、类风湿性关节炎、维生素D过多症和鳃骨生殖器综合征等潜在疾病的症状之一。1920年，巴尔施米德首次将其描述为无根牙，1939年，拉什顿将其命名为牙本质发育异常。它分为I型、II型和III型，其中III型仅影响恒牙列。本文报告了一例26岁男性患者罕见的I型牙本质发育异常病例，并重点关注临床、放射学、磨片和组织病理学方面。它强调了早期诊断和干预对个体心理健康的重要性。
