Background: Pyloric atresia is a rare but serious condition that can occur with junctional epidermolysis bullosa (PA-JEB). Early recognition is necessary for timely intervention, but prognosis can be serious and mortality is high. Objective: We describe the case of a patient with PA-JEB who not only survived past infancy but showed improvement in cutaneous blistering as she grew older. Conclusion: With early surgical intervention, some patients with PA-JEB can survive and look forward to a favourable prognosis, with improvement of cutaneous blistering by early childhood.
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