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Abstract：
Introduction: Epidermolysis bullosa (EB) is a genetically inherited disease characterized by recurrent bullae and erosions on the skin with numerous signs of dental caries and poor oral hygiene. The aim of this study was to investigate the general clinical and oral findings of patients with EB. Materials and Methods: In this prospective study, the clinical and oral findings and family history of 26 cases with EB were evaluated. The type of EB, gender, age, parental consanguinity, dental caries, oral findings, distribution of lesions and presence of associated anomalies, clinical and oral findings correlated with gender were recorded. Results: All 26 patients with EB had a history of consanguinity and siblings with EB to varying degrees. In our study, malnutrition, anemia and growth retardation, gastrointestinal system complications, hair thinning, hand and nail deformity, ocular problems and renal disease (in one case) were observed with variable frequencies. When the intraoral findings of the patients were investigated, extensive dental caries in all EB types, enamel hypoplasia in junctional EB (JEB) and the presence of tooth-root to be extracted in dystrophic EB (DEB), intraoral bullae and lesions, ankyloglossia, vestibular sulcus insufficiency, microstomia and maxillary atrophy were observed. Three cases had restorative treatment and one case had prosthetic rehabilitation. Conclusions: Oral involvement can be seen with varying frequencies depending on the type of EB and the severity of the disease. It may result from delayed oral and dental rehabilitation due to physical disabilities, limitations and more pressing medical problems. Microstomy, pain from mucosal lesions, and restricted access to the mouth can be caused by poor oral hygiene. Oral complications and caloric needs of individuals with EB should be determined, and individual prophylaxis should be applied to prevent caries formation and protect teeth.
摘要：
简介：大疱性表皮松解症（EB）是一种遗传遗传性疾病，其特征是反复出现的大疱性和皮肤糜烂，有许多龋齿和口腔卫生不良的迹象。这项研究的目的是调查EB患者的一般临床和口腔表现。材料和方法：在这项前瞻性研究中，评估了26例EB患者的临床和口腔检查结果以及家族史。EB的类型,性别,年龄,父母血缘关系,龋齿,口头发现，病变的分布和相关异常的存在，记录与性别相关的临床和口腔检查结果.结果：26例EB患者均有不同程度的血缘关系及兄弟姐妹史。在我们的研究中,营养不良,贫血和生长迟缓，胃肠系统并发症,头发稀疏,手和指甲畸形，观察到不同频率的眼部问题和肾脏疾病（在一例中）。当调查患者的口内发现时，所有EB类型的广泛龋齿，交界性EB（JEB）中的釉质发育不全和营养不良性EB（DEB）中的牙根存在，口腔内大疱和病变，强直,前庭沟功能不全,观察到小口和上颌萎缩。3例进行了恢复性治疗，1例进行了假肢康复。结论：根据EB的类型和疾病的严重程度，可以观察到不同频率的口腔受累。它可能是由于身体残疾导致的口腔和牙齿康复延迟，局限性和更紧迫的医疗问题。显微造口术,粘膜病变引起的疼痛，口腔卫生差可能会导致进入口腔的限制。应确定EB患者的口腔并发症和热量需求，和个别预防应用，以防止龋齿形成和保护牙齿。
