title: Mucous Membrane Pemphigoid Causing Central Airway Obstruction.
abstract: Mucous membrane pemphigoid (MMP) is a rare variant of the skin disease pemphigoid, which predominantly involves the mucous membranes. This rare autoimmune disease that infrequently affects the respiratory tract is characterized by subepithelial blister formation that may result in scarring. Immunopathologic examination of mucous membranes reveals the deposition of immunoglobulins and complement within the subepithelial basement membrane. We describe a patient with undiagnosed MMP, with a near-fatal presentation of central airway obstruction causing acute respiratory distress. The patient was successfully treated with emergent rigid bronchoscopic resection of a ball valve-like endotracheal mass, and diagnosed with a rare variant of pemphigoid disease, MMP. The patient was treated with mycophenolate and was clinically in remission, with bronchoscopically stable lesions at 1 year of follow-up.
authors.affiliation: *Department of Pulmonary, Critical Care & Sleep Disorders Medicine, University of Louisville, Louisville, KY Departments of †Internal Medicine §Pulmonary and Critical Care Medicine, University of New Mexico, Albuquerque ‡CHRISTUS St. Vincent Regional Medical Center, Santa Fe, NM.
authors.forename: Bilal A
authors.initials: BA
authors.lastname: Jalil
authors.forename: Yara G
authors.initials: YG
authors.lastname: Abdou
authors.forename: Stephanie A
authors.initials: SA
authors.lastname: Rosen
authors.forename: Tarek
authors.initials: T
authors.lastname: Dammad
mesh_terms: D000402:Airway Obstruction / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use; D001999:Bronchoscopy / Q000379:methods; D005260:Female; D006801:Humans; D009092:Mucous Membrane / Q000473:pathology; D009173:Mycophenolic Acid / Q000008:administration & dosage / Q000627:therapeutic use*; D010390:Pemphigoid, Benign Mucous Membrane / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D010391:Pemphigoid, Bullous / Q000473:pathology; D012128:Respiratory Distress Syndrome / Q000175:diagnosis / Q000209:etiology; D014132:Trachea / Q000276:immunology / Q000473:pathology / Q000601:surgery*; D014139:Tracheostomy / Q000379:methods; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000903:Antibiotics, Antineoplastic; D009173:Mycophenolic Acid