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Dental treatment in a patient with dystrophic epidermolysis bullosa under general anesthesia: A case report

대한장애인치과학회지 2020년 16권 1호 p.27 ~ 34
최원석, 이상호, 이난영, 김두수, 김지웅, 윤가람, 지명관,
소속 상세정보
최원석 ( Choi Won-Seok ) - Chosun University School of Dentistry Department of Pediatric Dentistry
이상호 ( Lee Sang-Ho ) - Chosun University School of Dentistry Department of Pediatric Dentistry
이난영 ( Lee Nan-Young ) - Chosun University School of Dentistry Department of Pediatric Dentistry
김두수 ( Kim Doo-Soo ) - Chosun University School of Dentistry Department of Pediatric Dentistry
김지웅 ( Kim Ji-Woong ) - Chosun University School of Dentistry Department of Pediatric Dentistry
윤가람 ( Yoon Ga-Ram ) - Chosun University School of Dentistry Department of Pediatric Dentistry
지명관 ( Jih Myeong-Kwan ) - Chosun University School of Dentistry Department of Pediatric Dentistry

Abstract


Epidermolysis Bullosa (EB) is a rare genetic disease that causes pain and results in peeling of the skin and mucous membranes, as well as easy blistering due to minor trauma or friction. EB is classified into four types according to the layer where the tissue is desquamated and blisters form. There are three genetic disorder-type EB diseases, namely, epidermolysis bullosa simplex (EBS), junctional epidermolysis bullosa (JEB), and dystrophic epidermolysis bullosa (DEB), as well as an acquired autoimmunity disorder. DEB shows autosomal recessive or dominant inheritance, and is characterized by deformities in the skin including coalescence of the fingers, blisters, scars, abnormalities of the fingertips, and milia. In extreme cases, a narrow esophagus or airway, ankyloglossia, microglossia, or squamous cell carcinoma may be seen. Dental problems associated with EB include microstomia or ankyloglossia, caused by scarring due to repeated blistering, as well as a high risk of dental caries due to difficulty in controlling a toothbrush (in turn due to hand and finger scarring). Therefore, continuous regular examination and preventive care are important. In this case report, we describe the dental treatment applied under general anesthesia for rampant caries in a 9-year-old patient with DEB who visited Chosun University Dental Hospital.

키워드

Ankyloglossia; Dental treatment; Dystrophic epidermolysis bullosa; General anesthesia; Microstomia

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