Title of article
Junctional Epidermolysis Bullosa Associated Laryngeal Stenosis: A Case Report and Review of Literature
Author/Authors
Balushi ، Firyal Otolaryngology Head and Neck Surgery Division, Surgery Department - Rustaq Hospital , Al Harrasi ، Ziyad Otolaryngology Head and Neck Surgery Division, Surgery Department - Rustaq Hospital , Al Farsi ، Maathir Otolaryngology Head and Neck Surgery Division, Surgery Department - Rustaq Hospital , Al Qurani ، Marwa Otolaryngology Head and Neck Surgery Division, Surgery Department - Rustaq Hospital
From page
613
To page
617
Abstract
Introduction: Junctional Epidermolysis Bullosa (JEB) is a rare subtype of the Epidermolysis Bullosa which itself is a rare genetic disorder. While mucosal involvement of pharynx and oesophagus has been reported, laryngeal involvement is rare. Case Report: A 7-month-old male child who was known to have Junctional Epidermolysis Bullosa presented to the emergency department with respiratory distress associated with a stridor which was eventfully found to have multiple level laryngeal stenosis. Conclusions: Longitudinal cohort studies are required to determine the long-term outcome and the anticipated behavior of epidermolysis bullosa in patients with laryngeal manifestation to avoid unnecessary surgical interventions.
Keywords
Airway , Junctional Epidermolysis Bullosa , Laryngeal Stenosis
Journal title
Iranian Journal of Otorhinolaryngology
Journal title
Iranian Journal of Otorhinolaryngology
Record number
2763266
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