PROTEIN-LOSING ENTEROPATHY IN A CHILD WITH JUNCTIONAL EPIDERMOLYSIS-BULLOSA AND PYLORIC ATRESIA

We report on a newborn boy with junctional epidermolysis bullosa and p yloric atresia. Blisters were found on his skin at birth, especially i n places exposed to pressure, and appeared later on his mucous membran es. Epidermolysis bullosa was confirmed by electron microscopy. Radiog raphy revealed pyloric atresia, and a gastroduodenostomy was carried o ut at 7 days of age. A connective tissue septum was found between his ventricle and duodenum. The skin changes were mild, and the clinical c ourse was determined by his protein-losing enteropathy. He died at 66 days of age from pseudomonas sepsis.