Meldgaard Lund A, Karlsmark T, Kobayasi T
Department of Pediatrics, Rigshospitalet, Copenhagen, Denmark.
Acta Derm Venereol. 1995 Jan;75(1):59-61. doi: 10.2340/00015555755961.
We report on a newborn boy with junctional epidermolysis bullosa and pyloric atresia. Blisters were found on his skin at birth, especially in places exposed to pressure, and appeared later on his mucous membranes. Epidermolysis bullosa was confirmed by electron microscopy. Radiography revealed pyloric atresia, and a gastroduodenostomy was carried out at 7 days of age. A connective tissue septum was found between his ventricle and duodenum. The skin changes were mild, and the clinical course was determined by his protein-losing enteropathy. He died at 66 days of age from pseudomonas sepsis.
我们报告了一名患有交界性大疱性表皮松解症和幽门闭锁的男婴。出生时其皮肤上发现有水泡，尤其是在受压部位，随后在其粘膜上也出现了水泡。通过电子显微镜检查确诊为大疱性表皮松解症。X线检查显示幽门闭锁，患儿在7日龄时接受了胃十二指肠吻合术。在其胃和十二指肠之间发现了结缔组织间隔。皮肤变化较轻，临床病程取决于其蛋白丢失性肠病。他在66日龄时死于假单胞菌败血症。
