Iranian Journal of Otorhinolaryngology

Iranian Journal of Otorhinolaryngology

Junctional Epidermolysis Bullosa Associated Laryngeal Stenosis: A Case Report and Review of Literature

Document Type : Case Report

Authors
Firyal Balushi
Ziyad Al Harrasi
Maathir Al Farsi
Marwa Al Qurani
Otolaryngology Head and Neck Surgery Division, Surgery Department, Rustaq Hospital.
10.22038/ijorl.2024.78360.3639
Abstract
Introduction:
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
Subjects
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Iranian Journal of Otorhinolaryngology
Volume 36, Issue 5
September and October 2024
Pages 613-617