Serrano Martínez C, Silvestre Donat F J, Bagán Sebastián J V, Peñarrocha Diago M, Alió Sanz J J
Departamento de Estomatología de la Universidad de Valencia, España.
Med Oral. 2001 Jan-Feb;6(1):48-56.
Hereditary epidermolysis bullosa (EB) is a mucocutaneous disorder characterized by the appearance of blisters and vesicles in response to minimum friction. The digestive mucosa is one of the most frequently affected regions--including the oral mucosa. Three types of EB have been established according to the histological level of the lesion. Thus, simple EB involves intraepidermal bullae that leave no scars, while junctional EB exhibit blisters between the lamina lucida and lamina densa of the basal membrane. These lesions heal leaving atrophy and involve important hypoplastic lesions in the dental enamel. In turn, dystrophic EB presents synechiae-forming subepidermal blisters--the recessive form being the variant involving the greatest oral lesions (microstomia, ankyloglossia, milium cysts and rampant caries). Three cases of EB are presented and their clinical-dental management difficulties are described. The oral manifestations are described, along with the dental treatments provided and the evolution of the periodontal indices over a two-year period following the application of hygiene-preventive and therapeutic measures.
遗传性大疱性表皮松解症（EB）是一种黏膜皮肤疾病，其特征是在受到最小摩擦时出现水疱。消化黏膜是最常受影响的区域之一，包括口腔黏膜。根据病变的组织学水平，已确定了三种类型的EB。因此，单纯性EB涉及表皮内水疱，不会留下疤痕，而交界性EB在基底膜的透明层和致密层之间出现水疱。这些病变愈合后会留下萎缩，并累及牙釉质的重要发育不全病变。反过来，营养不良性EB表现为形成粘连的表皮下水疱，隐性形式是涉及最大口腔病变（小口畸形、舌系带过短、粟丘疹囊肿和猖獗龋）的变体。本文介绍了3例EB病例，并描述了其临床牙科管理的困难。描述了口腔表现、提供的牙科治疗以及在应用卫生预防和治疗措施后两年内牙周指数的变化情况。
