Laryngoceles with airway compromise complicating mucous membrane pemphigoid

BMJ Case Rep. 2018 Jul 18:2018:bcr2018225316. doi: 10.1136/bcr-2018-225316.

Abstract

Mucous membrane pemphigoid (MMP) encompasses a group of autoantibody-mediated, subepithelial blistering diseases, which primarily affect mucosal surfaces including oral, ocular, skin, genital, nasopharyngeal and oesophageal sites. We present a first description of laryngoceles as a manifestation of mucous membrane pemphigoid resulting in dynamic airway closure. Mucosal injury at other sites had previously resulted in pathergy and localised cicatrisation. We discuss successful combined medical and transcutaneous surgical intervention designed to avoid tracheostomy and minimise iatrogenic laryngeal cicatrisation.

Keywords: ear, nose and throat/otolaryngology; immunology.

Publication types

  • Case Reports

MeSH terms

  • Airway Obstruction / etiology*
  • Antibiotics, Antineoplastic / therapeutic use
  • Humans
  • Laryngocele / etiology*
  • Laryngocele / therapy
  • Male
  • Middle Aged
  • Mycophenolic Acid / administration & dosage
  • Pemphigoid, Benign Mucous Membrane / complications*
  • Pemphigoid, Benign Mucous Membrane / therapy
  • Rituximab / administration & dosage

Substances

  • Antibiotics, Antineoplastic
  • Rituximab
  • Mycophenolic Acid