Stenosis of left mainstem bronchus in a case of cicatricial pemphigoid

A case of a 22-year-old male with cicatricial pemphigoid and involvement of the bronchial system is reported. Manifestations of cicatricial pemphigoid in the mucous membranes of the airway distal to the larynx have not been hitherto described. The patient developed severe stenosis of the left mainstem bronchus 2 years after onset of the disease. Successful treatment by sleeve resection and end-to-end anastomosis was achieved. Diagnostic bronchoscopy in patients with severe dyspnoea suffering from cicatricial pemphigoid is recommended. Operative treatment of airway stenoses distal to the larynx caused by cicatritial pemphigoid is feasible.