Ehlers-Danlos Syndrome (Type VIII)

抄録

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 den-tal 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.