Department of Orthodontics, IRCCS Cà Granda - Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
Oral Surg Oral Med Oral Pathol Oral Radiol. 2012 Aug;114(2):147-53. doi: 10.1016/j.oooo.2012.04.005.
Hyper-IgE syndrome (HIES) is a rare multisystem disorder with both immunologic and nonimmunological features. It is characterized by extremely elevated IgE serum levels, eczema, and recurrent skin and pulmonary infections. Dental anomalies are often included, such as retention of deciduous teeth together with ectopic eruption or noneruption of permanent teeth. Severe susceptibility to caries and mycotic infections, insufficient transversal diameter of the palate, mucosal plaques, and fissures typically located on the tongue and on the palate are often present. The aim of this study was to review the literature and to report a 6-year observation of 6 patients with HIES (aged 8-39 years) with focus on their oral problems and the treatment provided. The importance of the role of the dentist both in early diagnosis of this syndrome and in monitoring oral conditions was stressed. The dentist can prevent infective complications and intercept the development of malocclusion with a reduction of the need for complex treatment.
高免疫球蛋白 E 综合征（HIES）是一种罕见的多系统疾病，具有免疫和非免疫特征。其特征是血清 IgE 水平极度升高、湿疹以及反复的皮肤和肺部感染。常伴有牙齿异常，如乳牙滞留、恒牙异位或未萌出。此外，还常存在龋齿和真菌感染的高度易感性、腭横向直径不足、黏膜斑块以及典型位于舌和腭上的裂隙。本研究旨在回顾文献，并报告 6 例 HIES 患者（年龄 8-39 岁）的 6 年观察结果，重点关注他们的口腔问题和所提供的治疗。强调了牙医在早期诊断该综合征和监测口腔状况方面的重要作用。牙医可以预防感染性并发症，并通过减少复杂治疗的需要来阻止错颌的发展。
