Louloudiadis A K, Louloudiadis K A
Dept Preventive Dentistry, Dental School, Aristotle University, Thessaloniki, Greece.
Eur Arch Paediatr Dent. 2009 Jan;10(1):42-5. doi: 10.1007/BF03262667.
Epidermolysis Bullosa (EB) is a group of rare genetically determined disorders characterised by the development of blisters following minor or insignificant trauma or traction to the skin or mucosal surfaces. It appears that the cause of EB is related to abnormal enzyme activity and collagen degradation, but the exact pathogenesis and aetiology remain obscure. There are three major forms -- simple, junctional, and dystrophic. Oral manifestations and dental involvement of EB vary in frequency, and severity according to the form.
A 13-year old boy with a known diagnosis of Dystrophic Epidermolysis Bullosa (DEB) since birth was seen in our dental practice in 2004. Upon examination, the patient had multiple scars and blisters involving his neck, head, elbows and knees. Pseudosyndactyly was present on both hands. Intraoral examination revealed multiple carious lesions affecting almost the entire dentition, with severe gingival inflammation, mycrostomia, ankyloglossia and elimination of buccal and vestibular sulci.
This was divided into four phases- (1) oral hygiene and dietary instructions, elimination of gingival inflammation, (2) restorative procedures, (3) extractions of the carious destroyed teeth, and (4) recall system.
FOLLOW-UP: The patient was recalled on a schedule every three months with continued evaluation of oral status (Pl and GI indices). The preventive regimen included twice yearly topical fluoride application (Duraphat 2.26%). The final recommendations for our patient (except brushing twice a day), were to use daily fluoride mouthrinse (NaF 0.05%), to avoid cariogenic food products, and to visit a dietician to correct his diet.
By applying present knowledge of DEB the oral health of these patients can be improved and eliminate complications arising from the oral condition. After three routine check-ups and due to the cooperation of this patient, his oral health status remains very satisfactory.
大疱性表皮松解症（EB）是一组罕见的遗传性疾病，其特征是皮肤或粘膜表面受到轻微或不明显的创伤或牵拉后出现水疱。EB的病因似乎与酶活性异常和胶原蛋白降解有关，但其确切的发病机制和病因仍不清楚。EB主要有三种类型——单纯型、交界型和营养不良型。EB的口腔表现和牙齿受累情况在频率和严重程度上因类型而异。
一名自出生起就被诊断为营养不良型大疱性表皮松解症（DEB）的13岁男孩于2004年在我们的牙科诊所就诊。检查发现，患者颈部、头部、肘部和膝盖有多处瘢痕和水疱。双手均有假性并指。口腔检查发现多个龋损几乎累及全部牙列，伴有严重的牙龈炎症、小口畸形、舌系带过短以及颊沟和前庭沟消失。
治疗分为四个阶段——（1）口腔卫生和饮食指导，消除牙龈炎症；（2）修复治疗；（3）拔除龋坏牙齿；（4）回访制度。
患者每三个月定期回访，持续评估口腔状况（菌斑指数和牙龈指数）。预防措施包括每年两次局部应用氟化物（多乐氟2.26%）。对该患者的最终建议（除每天刷牙两次外）是每天使用含氟漱口水（0.05%氟化钠），避免食用致龋性食物，并咨询营养师调整饮食。
通过应用目前对DEB的认识，可以改善这些患者的口腔健康，消除口腔疾病引发的并发症。经过三次常规检查，由于该患者的配合，其口腔健康状况仍然非常令人满意。
