Doruk Cenk, Bicakci Ali Altug, Babacan Hasan
Faculty of Dentistry, University of Cumhuriyet, Sivas, Turkey.
Angle Orthod. 2003 Dec;73(6):763-8. doi: 10.1043/0003-3219(2003)073<0763:OAOTOA>2.0.CO;2.
Incontinentia pigmenti is an uncommon, inherited disorder with predominantly ectodermal manifestations that is associated with skin (100%)), dental (90%), skeletal (40%), central nervous (40%), and ocular (35%) deformities. It is an X-linked dominant disease, usually lethal in males and occurring in female infants. The dental effects include delayed eruption, partial anodontia, microdontia, and cone or peg-shaped teeth. The dental, clinical, and radiological findings in a 16-year-old female are presented here. The patient had peg-shaped teeth and a unilateral maxillary transverse discrepancy associated with oligodontia in the maxillary and mandibular arches. Orthodontic treatment included rapid maxillary expansion and fixed orthodontic therapy for prosthetic purposes and elimination of the functional midline shift.
色素失禁症是一种罕见的遗传性疾病，主要表现为外胚层异常，与皮肤（100%）、牙齿（90%）、骨骼（40%）、中枢神经（40%）和眼部（35%）畸形有关。它是一种X连锁显性疾病，通常对男性致死，多见于女婴。牙齿方面的影响包括萌出延迟、部分无牙症、小牙症以及锥形或钉状牙。本文介绍了一名16岁女性的牙齿、临床和放射学表现。该患者有钉状牙，上颌单侧横向差异，伴有上颌和下颌牙弓少牙症。正畸治疗包括快速上颌扩弓以及为修复目的和消除功能性中线偏移而进行的固定正畸治疗。
