Author(s)
Dennis Tang
Paul C. Bryson
Affiliation(s)
Cleveland Clinic Foundation
Abstract:
Objective: Mucous membrane pemphigoid (MMP) is a rare autoimmune disease characterized by sub-epithelial blistering. Cicatricial scar and inflammation involving the larynx and upper airway is a serious complication of MMP. We present a rare case of MMP complicated by stenosis of the nasal valve, velopharynx, supraglottis, and glottis requiring multiple surgical interventions. Method: Case Report Results: Patient is a 68 yo female with multiple oral, nasal, and laryngopharyngeal ulcers consistent with MMP. She was found to have significant unilateral nasal stenosis, supraglottic stenosis secondary to cicatricial scar, interarytenoid fixation, and active ulcerations. An awake tracheostomy was performed to secure her airway. Immunosuppressants were started resulting in remission. However, she continued to have multilevel stenosis. On examination, she was found to have a pinpoint supraglottic opening and underwent lyses of adhesions and dilation of her supraglottis and glottis. Her nasal cavity was stented with a Doyle split. These maneuvers established an acceptable airway but she continued to have velopharyngeal stenosis. A pharyngoplasty was performed with placement of a nasal trumpet in addition to repeat supraglottic dilation and application of mitomycin C. At 10 month follow-up, she has maintained decannulation and a patent upper airway with minimal restenosis of the velopharynx. Conclusion: MMP is a rare autoimmune condition that can have devastating consequences in the upper airway. This is a unique case of MMP involving 4 subsites requiring multiple interventions to restore airway patency. Effective immunosuppression and collaboration with rheumatology is critical to controlling the disease to allow for airway interventions.