• 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