Epidermolysis bullosa letalis associated with congenital pyloric atresia

A 32-week-old (gestational age) female infant with epidermolysis bullosa letalis (EBL) (confirmed by light and electron microscopy) had a gastric-outlet obstruction on routine roentgenographic examination. An autopsy showed a fibrous cord connecting the stomach and first part of the duodenum in the area of the pylorus. A review of the literature indicated 12 additional cases of epidermolysis bullosa (EBL where type was confirmed) associated with pyloric atresia. The possibility of coexistent pyloric atresia should be considered in a newborn who has suspected EBL.