Epidermolysis bullosa with associated pyloric atresia (EB-PA) is a rare subtype of epidermolysis bullosa with a high mortality characterized by skin fragility, pyloric atresia, as well as renal and ureteral abnormalities. We present a unique case of EB-PA in a newborn male further complicated by esophageal atresia and nephrotic syndrome. We review the previously reported nine cases of EB-PA with esophageal atresia.
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