Mucous membrane pemphigoid: nasal and laryngeal manifestations

J Laryngol Otol. 2003 Nov;117(11):885-8. doi: 10.1258/002221503322542917.

Abstract

Mucous membrane pemphigoid (MMP) is a sub-epithelial blistering disease that primarily involves mucosal surfaces but may also involve the skin. Clinically, it appears as vesiculobullous lesions of the oral cavity and eyes, but other tissues such as the nasopharyngeal and laryngeal mucosa can also be affected. Ultimately, scarring and airway stenosis may occur. The condition should be managed by a multidisciplinary team led by a dermatologist. Immunosuppressive therapy forms the mainstay of treatment, with surgery having both a diagnostic role and a use in the treatment of complications such as airway obstruction. There must be a low threshold of suspicion for laryngeal involvement in this group of patients, so that prompt action can be taken. Once laryngeal stenosis has occurred repeated endoscopic laser excision of scar tissue can be used to maintain an adequate airway. Adjuvant use of mitomycin-C can be used beneficially in the treatment of laryngeal complications of MMP.

Publication types

  • Case Reports

MeSH terms

  • Adjuvants, Immunologic / therapeutic use
  • Adult
  • Airway Obstruction / etiology
  • Blister / etiology
  • Female
  • Hand Dermatoses / etiology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Laryngeal Diseases / etiology*
  • Laryngoscopy
  • Laryngostenosis / etiology
  • Larynx / surgery
  • Laser Therapy / methods
  • Mitomycin / therapeutic use
  • Nose Diseases / etiology*
  • Pemphigoid, Benign Mucous Membrane / complications*
  • Pemphigoid, Benign Mucous Membrane / drug therapy
  • Pemphigoid, Benign Mucous Membrane / surgery

Substances

  • Adjuvants, Immunologic
  • Immunosuppressive Agents
  • Mitomycin