Maxillofacial Plastic and Reconstructive Surgery

Korean Association of Maxillofacial Plastic and Reconstructive Surgeons (대한악안면성형재건외과학회)
권호 표지

Epidermolysis Bullosa with Mouth Opening Limitation

개구제한을 동반한 수포성 표피박리증

  • Yun, Yeong-Eun (Department of Oral and Maxillofacial Surgery, Sun Dental Hospital) ;
  • Kim, Jae-Young (Department of Oral and Maxillofacial Surgery, Sun Dental Hospital) ;
  • Lee, Dong-Keun (Department of Oral and Maxillofacial Surgery, Sun Dental Hospital) ;
  • Kang, Ji-Yeon (Department of Oral and Maxillofacial Surgery, Sun Dental Hospital)
  • 윤영은 (선치과병원 구강악안면외과) ;
  • 김재영 (선치과병원 구강악안면외과) ;
  • 이동근 (선치과병원 구강악안면외과) ;
  • 강지연 (선치과병원 구강악안면외과)
  • Received : 2011.10.14
  • Accepted : 2011.11.23
  • Published : 2012.03.30
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Abstract

Epidermolysis bullosa (EB) represents a spectrum of conditions that are characterized by blistering and mechanical fragility of the skin. There is genetic heterogeneity and marked variation in clinical phenotypes in the multiple EB disorders. The most recent classification recognizes four major EB grouping and over 30 EB subtypes. The severity of EB ranges from mild to severe skin involvement, and it can be localized or generalized. Oral features include repeated occurrence of blisters, erosions, and scars, which lead to limited mouth opening, ankyloglossia, elimination of buccal vestibule, and increased risk of oral carcinoma. Routine dental care or even normal tooth brushing might cause bullae on the oral mucosa. Occasionally, the clinician will be called upon to treat patients with EB and should therefore be aware of specific treatment modifications. we present a reviews of the literature with a case providing adequate anesthetic and surgical care.

Keywords

References

  1. Fine JD, Eady RA, Bauer EA, et al. The classification of inherited epidermolysis bullosa (EB): report of the third interational consensus meeting on diagnosis and classification of EB. J Am Acad Dermatol 2008;58:931-50. https://doi.org/10.1016/j.jaad.2008.02.004
  2. Fine JD, Bauer EA, McGuire J, Moshell A, editors. Epidermolysis bullosa: clinical, epidemiologic, and laboratory advances and the findings of the national epidermolysis bullosa registry. Baltimore, Md: Johns Hopkins University Press; 1999.
  3. Koebner H. Hereditare anlage zur blasenbildung (epidermolysis bullosa hereditaria). Dtsch Med Wochenschr 1886;12:21-2. https://doi.org/10.1055/s-0028-1139665
  4. Pearson RW. Studies on the pathogenesis of epidermolysis bullosa. J Invest Dermatol 1962;39:551-75. https://doi.org/10.1038/jid.1962.156
  5. Has C. Molecular genetic assays for inherited epidermolysis bullosa. Clin Dermatol 2011;29:420-6. https://doi.org/10.1016/j.clindermatol.2011.01.011
  6. Okulicz JF, Kihiczak NI, Janniger CK. Epidermolysis bullosa simplex. Cutis 2002;70:19-21.
  7. Yan EG, Paris JJ, Ahluwalia J, Lane AT, Bruckner AL. Treatment decision-making for patients with the Herlitz subtype of junctional epidermolysis bullosa. J Perinatol 2007; 27:307-11. https://doi.org/10.1038/sj.jp.7211694
  8. Das BB, Sahoo S. Dystrophic epidermolysis bullosa. J Perinatol 2004;24:41-7. https://doi.org/10.1038/sj.jp.7211019
  9. White SJ, McLean WH. Kindler surprise: mutations in a novel actin-associated protein cause Kindler syndrome. J Dermatol Sci 2005;38:169-75. https://doi.org/10.1016/j.jdermsci.2004.12.026
  10. Price-Douglas W, Diehl-Svrjcek B. Epidermolysis bullosa: a case study in transport, treatment, and care. Adv Neonatal Care 2007;7:289-94. https://doi.org/10.1097/01.ANC.0000304967.46708.ea
  11. McGrath JA, Schofield OM, Mayou BJ, McKee PH, Eady RA. Epidermolysis bullosa complicated by squamous cell carcinoma: report of 10 cases. J Cutan Pathol 1992;19:116-23. https://doi.org/10.1111/j.1600-0560.1992.tb01352.x
  12. Wessagowit V, Chunharas A, Wattanasirichaigoon D, McGrath JA. Globalization of DNA-based prenatal diagnosis for recessive dystrophic epidermolysis bullosa. Clin Exp Dermatol 2007;32:687-9. https://doi.org/10.1111/j.1365-2230.2007.02510.x
  13. McGrath JA, Mellerio JE. Epidermolysis bullosa. J Med Archives Vol 2011;24:74-87.
  14. Penarrocha-Oltra D, Penarrocha-Diago M, Balaguer-Martinez J, Ata-Ali J, Penarrocha-Diago M. Full-arch fixed prosthesis supported by four implants in patients with recessive dystrophic epidermolysis bullosa. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011;112:e4-10.
  15. Lee H, Al Mardini M, Ercoli C, Smith MN. Oral rehabilitation of a completely edentulous epidermolysis bullosa patient with an implant-supported prosthesis: a clinical report. J Prosthet Dent 2007;97:65-9. https://doi.org/10.1016/j.prosdent.2006.12.010
  16. Iohom G, Lyons B. Anaesthesia for children with epidermolysis bullosa: a review of 20 years' experience. Eur J Anaesthesiol 2001;18:745-54.