Junctional epidermolysis bullosa with pyloric atresia is a severe subtype of junctional epidermolysis bullosa (JEB) characterized by generalized blistering at birth and congenital atresia of the pylorus and rarely of other portions of the gastrointestinal tract. The disease is MONDO_0009183 (junctional epidermolysis bullosa with pyloric atresia). Also known as: Carmi syndrome, JEB-PA, epidermolysis bullosa junctionalis with pyloric atresia, epidermolysis bullosa, junctional, with pyloric stenosis, junctional epidermolysis bullosa-pyloric atresia syndrome.