Açikgöz Aydan, Kademoglu Oya, Elekdag-Türk Selma, Karagöz Filiz
Department of Oral Diagnosis and Radiology, Faculty of Dentistry, University of Başkent, Ankara, Turkey.
Quintessence Int. 2007 Nov-Dec;38(10):853-8.
Ectodermal dysplasia is a rare congenital hereditary entity. The most commonly observed forms of ectodermal dysplasia are the hidrotic and hypohidrotic types; discrimination is based on the absence or presence of sweat glands. In this report, a child with hypohidrotic ectodermal dysplasia having true anodontia of the primary dentition is presented. Physical, mental, and intraoral examinations were performed. Photographs and occlusal and panoramic radiographs were taken. A medical consultation and biopsies were requested. Mentally, the patient was normal. His hair and eyebrows were light colored and sparse. He had frontal bossing, a saddle nose, reduced vertical facial dimension, and prominent supraorbital ridges and chin. Radiographs revealed absence of all primary and permanent teeth except the bilaterally unerupted maxillary permanent canines. An armpit biopsy specimen revealed the absence of eccrine and apocrine glands. A removable prosthesis was made to satisfy the patient's esthetic and functional needs. The absence of primary teeth (true anodontia) is a rare phenomenon. It is claimed that primary teeth must be present for the development of their permanent successors. In the present case, however, the permanent canines existed despite the absence of their predecessors. Dental clinicians can be the first to diagnose ectodermal dysplasia. The dental team should be aware of its signs and symptoms in order to provide the correct therapies for the functional and psychologic needs of these patients.
外胚层发育不全是一种罕见的先天性遗传性疾病。最常见的外胚层发育不全类型是出汗型和少汗型；区分基于汗腺的有无。在本报告中，介绍了一名患有少汗型外胚层发育不全且乳牙真正无牙症的儿童。进行了体格、心理和口腔内检查。拍摄了照片、咬合片和全景片。请求进行医学咨询和活检。心理方面，患者正常。他的头发和眉毛颜色浅且稀疏。他有额部隆起、鞍鼻、面部垂直尺寸减小、眶上嵴和下巴突出。X线片显示除双侧未萌出的上颌恒牙尖牙外，所有乳牙和恒牙均缺失。腋窝活检标本显示无小汗腺和大汗腺。制作了可摘义齿以满足患者的美观和功能需求。乳牙缺失（真正无牙症）是一种罕见现象。据称乳牙必须存在才能发育其恒牙后继者。然而，在本病例中，尽管恒牙尖牙的乳牙缺失，但恒牙尖牙却存在。牙科临床医生可能是最早诊断外胚层发育不全的人。牙科团队应了解其体征和症状，以便为这些患者的功能和心理需求提供正确的治疗方法。
