Apaydin A
Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, University of Istanbul, Turkey.
J Nihon Univ Sch Dent. 1995 Dec;37(4):214-7. doi: 10.2334/josnusd1959.37.214.
Ehlers-Danlos syndrome (EDS), a group of rare, autosomal dominantly inherited connective tissue dysplasias, characterized mainly by abnormal collagen synthesis, has been shown to exhibit extensive heterogeneity with at least 11 clinical entities differentiated by their clinical, biochemical, and genetic features. Of these, Type VIII EDS is of special interest from a dental viewpoint, due mainly to the resulting fragility of the oral mucosa and blood vessels, and an aggressive type of periodontitis causing severe loss of alveolar bone; all of these clinical manifestations of the entity make it necessary to exercise care during the dental treatment procedures. Here we present a case of EDS Type VIII with typical clinical findings, together with details of the dental treatment modalities, placing emphasis on surgical methods.
埃勒斯-当洛综合征(EDS)是一组罕见的常染色体显性遗传结缔组织发育异常疾病,主要特征为胶原蛋白合成异常,已显示出广泛的异质性,至少有11种临床类型,可通过其临床、生化和遗传特征进行区分。其中,从牙科角度来看,VIII型EDS特别值得关注,主要是因为它会导致口腔黏膜和血管脆弱,以及一种侵袭性牙周炎,导致牙槽骨严重丧失;该疾病的所有这些临床表现使得在牙科治疗过程中必须格外小心。在此,我们报告一例具有典型临床表现的VIII型EDS病例,并详细介绍牙科治疗方式,重点是手术方法。