Tosun Gul, Elbay Ulku
Department of Pediatric Dentistry, Faculty of Dentistry, Selçuk University Konya, Turkey.
J Clin Pediatr Dent. 2009 Fall;34(1):71-5. doi: 10.17796/jcpd.34.1.kr015833p1qg6873.
Rapp-Hodgkin syndrome is a rare form of ectodermal dysplasia involving the hair, eyes, sweat glands, nails, teeth and palate. This syndrome is characterized by stiff sparse hair with the appearance of steel wool, sparse eyebrows and lashes, cleft palate, absence of lacrimal punctae, epiphora, a decreased number of sweat glands, and dystrophic nails. Cleft palate, hypodontia, abnormal tooth shape, multiple caries, delayed eruption of teeth are the main oral manifestations. In this paper we describe the clinical and dental findings of this syndrome in a 7 year old girl referred to the dental clinic because of severe tooth ache. The dental treatment included root canal treatment, composite restorations, extractions and removable prostheses. This paper highlights features of Rapp-Hodgkin Syndrome and its dental rehabilitation.
拉普-霍奇金综合征是一种罕见的外胚层发育不良，累及毛发、眼睛、汗腺、指甲、牙齿和腭部。该综合征的特征为毛发僵硬稀疏，呈钢丝绒样外观，眉毛和睫毛稀疏，腭裂，泪点缺失，溢泪，汗腺数量减少以及指甲营养不良。腭裂、牙齿发育不全、牙齿形状异常、多发龋齿、牙齿萌出延迟是主要的口腔表现。本文描述了一名7岁女童因严重牙痛转诊至牙科诊所，该综合征的临床和牙科检查结果。牙科治疗包括根管治疗、复合树脂修复、拔牙和可摘义齿修复。本文重点介绍了拉普-霍奇金综合征的特征及其牙科康复治疗。
