Case Reports
Junctional Epidermolysis Bullosa with Pyloric Atresia: A Case with Favourable Outcome
Duc Ha,* Halliday Idikio,† Alfons Krol,* and Andrew N. Lin*
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.
Antécédents: L'atrésie du pylore est une maladie rare mais grave, parfois concomitante de l'épidermolyse bulleuse jonctionnelle (PA-JEB). Le diagnostic doit être rapide pour permettre une intervention opportune, mais le pronostic reste préoccupant et le taux de mortalité élevé.
Objectif: Décrire le cas d'un patient souffrant de PA-JEB qui a non seulement survécu au-delà de la première enfance mais chez qui la vésication s'est atténuée avec l'âge.
Conclusion: Une intervention chirurgicale précoce chez ces patient assure parfois leur survie, et ils peuvent envisager un pronostic favorable, l'apparition de bulles allant en diminuant au début de l'enfance.
Received 5/26/98. Accepted for publication 7/14/98.
*Division of Dermatology and Cutaneous Sciences, University of Alberta, Edmonton, Alberta, Canada and †Department of Laboratory Medicine and Pathology, University of Alberta, Edmonton, Alberta, Canada
Duc Ha is a medical student at the University of Saskatchewan, Saskatoon, Saskatchewan, doing a dermatology elective at the University of Alberta, Edmonton, Alberta.
Reprints not available from the author.
Address for correspondence: Dr. Andrew N. Lin, University Dermatology Centre, 2-104 Clinical Sciences Building, University of Alberta, Edmonton, Alberta T6G 2G3, Canada
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