Torres Carolina Paes, Gomes-Silva Jaciara Miranda, Mellara Thalita Siqueira, Carvalho Lívia Pasqualini, Borsatto Maria Cristina
Department of Pediatric Clinic, Preventive and Community Dentistry, Ribeirão Preto Dental School, University of São Paulo, Ribeirão Preto, SP, Brazil.
Braz Dent J. 2011;22(6):511-6. doi: 10.1590/s0103-64402011000600012.
Epidermolysis bullosa (EB) is a heterogeneous group of rare genetic disorders characterized by marked fragility of the skin and mucous membranes in which vesiculobullous lesions occur in response to trauma, heat or no apparent cause. The recessive form of EB presents the greatest oral alterations including repeated blistering and scar formation leading to limited oral opening, ankyloglossia, tongue denudation, microstomia, vestibule obliteration and predisposition to oral carcinoma. Routine dental care may cause bullae formation on the lips and oral mucosa. Together with the ingestion of soft and frequently carbohydrate food, these anomalies lead to a high caries risk. This paper documents a case of a child diagnosed with recessive dystrophic epidermolysis bullosa (RDEB); describes the phases and difficulties of dental treatment and the measures that dentists and health care providers should adopt in order to provide a safe and effective dental treatment as well as earlier prevention to these patients.
大疱性表皮松解症（EB）是一组罕见的遗传性疾病，其特征是皮肤和黏膜极度脆弱，在受到创伤、热刺激或无明显诱因时会出现水疱性大疱性病变。EB的隐性形式会导致最严重的口腔改变，包括反复水疱形成和瘢痕形成，导致张口受限、舌系带过短、舌部剥脱、小口畸形、前庭闭锁以及口腔癌易感性增加。常规牙科护理可能会导致嘴唇和口腔黏膜形成水疱。再加上摄入柔软且富含碳水化合物的食物，这些异常情况会导致患龋齿的风险很高。本文记录了一例被诊断为隐性营养不良性大疱性表皮松解症（RDEB）的儿童病例；描述了牙科治疗的阶段和困难，以及牙医和医疗保健提供者为这些患者提供安全有效的牙科治疗和早期预防应采取的措施。
