The Journal of Korea Assosiation for Disability and Oral Health (대한장애인치과학회지)

Korean Association for Disability and Oral Health (대한장애인치과학회)
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DENTAL TREATMENT OF PEDIATRIC EPIDERMOLYSIS BULLOSA PATIENTS : CASE REPORTS

수포성 표피 박리증 환자의 치과적 관리 : 증례보고

  • Han, Eun-Ok (Department of Pediatric Dentistry, School of Dentistry, Pusan National University) ;
  • Kim, Seong Hee (Department of Pediatric Dentistry, School of Dentistry, Pusan National University) ;
  • Kim, Shin (Department of Pediatric Dentistry, School of Dentistry, Pusan National University) ;
  • Jeong, Tae-Sung (Department of Pediatric Dentistry, School of Dentistry, Pusan National University)
  • 한은옥 (부산대학교 치의학전문대학원 소아치과학교실) ;
  • 김성희 (부산대학교 치의학전문대학원 소아치과학교실) ;
  • 김신 (부산대학교 치의학전문대학원 소아치과학교실) ;
  • 정태성 (부산대학교 치의학전문대학원 소아치과학교실)
  • Received : 2012.11.14
  • Accepted : 2012.12.03
  • Published : 2012.12.31
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Abstract

Epidermolysis bullosa (EB) is a genetic disorder, characterized by blisters on skin and mucosal surfaces even upon light mechanical damage. EB is caused by genetic mutations in at least seven proteins on the basement membrane zone, which is the boundary between the epidermis and the dermis. There are many types of EB differing in clinical and genetic aspects, and the prognosis varies depending on the EB type. There are largely three types of EB, categorized by the electron-microscopic location of the blisters. The blisters form within the epidermis in epidermolysis bullosa simplex, in the lamina lucida in junctional epidermolysis bullosa, and just beneath the basal lamina in dystrophic epidermolysis bullosa. To date, there is no medication or treatment that cures EB or completely prevents the blisters, so generally symptomatic treatment is performed. EB patients must always be cautious, for blisters can form at the slightest injuries, and the patients must be dealt with gently. Injuries and infections have to be prevented and treated, and deficient nutrients must be supplied during dental treatment period. Some patients may experience pain when swallowing food or dental treatment due to blisters and resulting scars in the mouth, pharynx, and esophagus. Recently, two pediatric patients were diagnosed with EB at Pusan National University Hospital and visited the Department of Pediatric Dentistry for oral care and dental treatment. The treatment results are reported here.

수포성 표피 박리증을 가진 환자들은 구강내 수포 형성, 연조직 점막의 박리, 개구 제한 등의 이유로 치과치료를 시행하는데 있어 상당한 어려움이 있다. 그러므로 치과 치료시 많은 주의사항이 필요하며, 무엇보다 치과 질환의 예방이 중요하다. 수포성 표피 박리증 환자에 있어 이른 시기부터의 구강 관리는 기능적 치열을 유지시켜 주어 저작 효율을 높여주고 영양 공급 면에서도 도움을 주어 성장 발육을 돕는다. 따라서 지속적인 식이 상담과 구강 위생 관리 교육, 불소 도포 등을 위한 정기 검진이 수포성 표피 박리증 환자에서는 필수적이다.

Keywords

References

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