Pyloric atresia and epidermolysis bullosa: A fatal association

Pyloric atresia (PA) contributes less than 1% of all gastrointestinal atresias with an incidence of 1 in 100,000 newborns. It is often associated with epidermolysis bullosa (EB) or aplasia cutis congenita. This association is believed to be lethal because of the increased risk of developing septicemia, electrolyte imbalance, and protein loss. Here, we attempt to highlight the fatal complications of PA with EB in a 10-day-old female baby and reevaluate the representative features that an astute clinician needs to be aware of.