Bentolila R, Rivera H, Sanchez-Quevedo M C
Pedodontics Graduate Program, Faculty of Dentistry, Central University of Venezuela, Caracas, Venezuela.
Pediatr Dent. 2006 Jan-Feb;28(1):54-7.
Incontinentia pigmenti (IP) is a genodermatosis with an X-linked dominant mode of inheritance, characterized by ectodermal, mesodermal, neurological, ocular, and dental manifestations. The purpose of this case study was to report the oral and dental manifestations of an IP case in a Venezuelan pediatric patient. A 9 year-old Venezuelan girl was evaluated. She showed macular pigmented lesions in her face, trunk, back, legs, and fingers as well as abnormal hair distribution, alopecia on the vertex, and hypoplasia of eyebrows. During the dental examination, conical shaped-teeth and delayed dental eruption was evidenced. The microanalytical examination showed dentin without significant alterations in the mineralization except for hypermineralization in focal areas. In addition, a decrease in the enamel mineralization was observed.
色素失禁症（IP）是一种具有X连锁显性遗传模式的遗传性皮肤病，其特征为外胚层、中胚层、神经、眼部和牙齿表现。本病例研究的目的是报告一名委内瑞拉儿科患者IP病例的口腔和牙齿表现。对一名9岁的委内瑞拉女孩进行了评估。她的面部、躯干、背部、腿部和手指出现了斑状色素沉着病变，以及毛发分布异常、头顶脱发和眉毛发育不全。在牙科检查中，发现了锥形牙和牙齿萌出延迟。微观分析检查显示，牙本质除局部区域矿化过度外，矿化无明显改变。此外，还观察到釉质矿化减少。
