위유문부 폐쇄증을 동반한 접합부성수포성표피박리증
Junctional Epidermolysis Bullosa (JEB) with Pyloric Atresia
이상협, 조용훈, 김해영,
소속 상세정보
이상협 ( Lee Sang-Hyup )
부산대학교 의과대학 외과학교실
조용훈 ( Cho Yong-Hoon )
부산대학교 의과대학 외과학교실
김해영 ( Kim Hae-Young )
부산대학교 의과대학 외과학교실
KMID : 0371320040670030244
Abstract
Epidermolysis bullosa (EB) has three distinctive types, of which junctional EB has been known to be associated with pyloric atresia (PA) usaually. PA is a congenital disease of gastric outlet obstruction, known to occur at a rate of 1 per million live-births. It may occur independently or in combination with other congenital disorders such as EB. Dozens of cases of this combined disease have been reported since 1972 and autosomal recessive patterns have recently been revealed. This, junctional epidermolysis bullosa with pyloric atresia, has poor prognosis. Bullous, erupted cutaneous manifestations have recurrent, persistent characteristics and causes extracutaneous manifestations such as electrolyte imbalance, protein-loosing enteropathy, failure to thrive, and sepsis. In some cases, this syndrome may have obstructive uropathy and give it bad results also. For these reasons, physicians should be careful to avoid minor trauma on the skin and need to make meticulous decisions in operative correction about obstrutive gastrointestinal lesion or uropathy. We report a case of 3-day-old male baby, junctional epidermolysis bullosa with pyloric atresia.
키워드
접합부성수포성표피박리증; 유문부 폐쇄증; Junctional epidermolysis bullosa; Pyloric atresia
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