Abstract
Epidermolysis bullosa (EB) is an inherited skin disease characterized by blister formation of the skin following just minor trauma or spontaneously. The main classification of EB is based on the localization of the blistering. In epidermolysis bullosa simplex, the blister formation is intraepidermal; in epidermolysis bullosa junctionalis the blister formation in the epidermal-dermal junction; in the dystrophic forms of EB blister formation is intradermal. Oral manifestations of recessive dystrophic EB are characterized by rampant caries, crowding, microstomia, ankyloglossia, vestibular obliteration. Dental therapy may be well provided to patient with recessive dystrophic EB by using general anesthesia especially in case of poor cooperation. A two years old girl with recessive dystrophic EB visited our clinic for rampant caries. She was hospitalized for severe anemia and fever, and we provided dental therapy under general anesthesia. Extraction of incisors and stainless steel crown restoration of primary molars were performed.
본 증례는 열성 이영양성 수포성 표피박리증을 가진 2세 8개월 된 환자로 다발성 우식증 소견을 보여 전신마취하에 치료하였다. 1. 환자는 열성 이영양성 수포성 표피박리증으로 전신에 흉터가 있었으며 심한 빈혈 및 패혈증 증상이 있어 수혈 및 항생제 등으로 먼저 전신상태를 회복하였다. 2. 전신마취하에 모든 절치의 발치 및 구치 부위의 치수치료와 기성금속관 수복을 시행하였다.