表皮分解性水泡症之牙齒異常－病例報告

Dental Anomalies in Epidermolysis Bullosa-Case Report

鄭安芝(An-Chih Cheng) ； 盧幼情(Yo-Chin Lu) ； 劉季煒(Ji-Uei Liou)

《中華牙醫學雜誌（中文版）》 25卷3期 (2006/09) Pp. 213-219

https://doi.org/10.7106/CJD.200609.0213

摘要

遺傳性表皮分解性水泡症（epidermolysis bullosa，簡稱EB）為一種罕見的遺傳性疾病，由於基因變異，只需手指觸碰的壓力，便可導致表皮層或真皮層的細胞分離，產生水泡或血泡，所以此種病患的皮膚非常脆弱。其嚴重程度根據不同的亞型而有不同程度的表現。本病例報告是一名十五歲女性，病理報告診斷為接合性表皮分解性水皰症(junctional epidermolysis bullosa)，口腔內診斷為全口牙釉質形成不良，但並無明顯口內產生水泡和結痂，導致口腔構造變形的情況，建議患者於上下顎前牙區，以直接複合樹脂貼片作為提供暫時性的美觀，後牙區以黏著式金屬咬合面冠蓋體，來維持咬合面高度避免牙本質過度磨耗，並降低患者牙齒敏感的程度，等待成年後牙冠長度夠長後，再進行全口重建。

關鍵字

表皮分解性水皰症 ； 牙釉質發育不全

並列摘要

Hereditary epidermolysis bullosa is a rare chronic mechanobullous group of genetic disorders mainly affecting the skin, and less frequently the oral and other mucosa. On the basis of the anatomical level of blister formation, the clinical features, the pattern of inheritance, and the expression of specific basement membrane features, the disease is classified into 3 major types and numerous subtypes. This case report is a 15-year-old female. The result of the pathological examination was junctional epidermolysis bullosa. No bulla or scarring was noted in the oral cavity, but all the dentition was characterized by enamel hypoplasia. Direct composite veneer was suggested for the maxillary and mandibular anterior teeth to enhance the esthetics and reduce the sensitivity of the dentition. An adhesive casting onlay was suggested for all of the posterior teeth, especially the first molar to maintain the vertical height of the clinical crown. Full-mouth rehabilitation will be suggested after the patient has completely grown up.

並列關鍵字

enamel hypoplasia ； epidermolysis bullosa