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Updated: May 27, 2026

Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
Upper airway complications of junctional epidermolysis bullosa
Jonathan B Ida1, Irina Livshitz, Richard G Azizkhan
1Division of Pediatric Otolaryngology, Head & Neck Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Objective:
To assess the incidence of upper airway pathology in patients with junctional epidermolysis bullosa (JEB).
Study Design:
We conducted a retrospective chart review of all patients with JEB who came to an interdisciplinary epidermolysis bullosa center at a tertiary care institution between 2004 and 2010.
Results:
Twenty-five patients with JEB were identified, and 12 patients were seen in the otolaryngology clinic (age range, 2 months-15 years; 8 male, 4 female). Of the 12 patients, 8 underwent rigid laryngoscopy and bronchoscopy for upper respiratory tract symptoms; 7 of these patients displayed laryngeal pathology, and 5 of them underwent surgical intervention with successful resolution of symptoms. Furthermore, none of these patients had any short- or long-term complications from their surgery. A strict protocol and a precise problem-focused cold surgical technique were used in these cases to protect skin and mucus membranes.
Conclusion:
With appropriate precautions, endoscopic laryngeal surgery can be safe and effective in patients with JEB and larygnotracheal disease. Endoscopic laryngeal surgery is feasible when indicated for these patients.
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