식도협착을 동반한 열성퇴행위축물집표피박리증 1예 |
김재왕 |
제주대학교 의학전문대학원 피부과학교실 |
Recessive dystrophic epidermolysis bullosa showing esophageal stricture |
Jae-Wang Kim |
Department of Dematology, Jeju National University School of Medicine, Jeju, Korea |
Correspondence:
Jae-Wang Kim, Email: rulid@jejunu.ac.kr |
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Abstract |
Dystrophic epidermolysis bullosa(DEB) is characterized by frequent erosive hemorrhagic bulla, scarring, malformation of hair, nail and tooth, acral deformity and considerable morbidity. Especially, DEB with recessive inheritance(RDEB) invariably discloses more extensive skin involvement and extracutaneous manifestation such as esophageal stricture or squamous cell carcinoma than in dominant DEB(DDEB). To the best of our knowledge, cases of RDEB accompanied by esophageal complications have been rarely reported in Korea. Herein, we report a rare case of EDEB showing esophageal fistula and stricture formation. |
Key Words:
Dystrophic epidermolysis bullosa; Esophageal stricture |
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