Mucous membrane pemphigoid: nasal and laryngeal manifestations

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.

Unusual association of diseases/symptoms

“…Herpes zoster, herpes simplex and varicella should be considered as readily treatable pathogens. Wegener's granulomatosis and Behçet's may also present with chronic granulomatous changes on both mucous membranes and skin and may be difficult to differentiate from cicatricial pemphigoid 4. In this case, we note the medical history to include ulcerative colitis.…”

“…There is no geographic or ethnic variation. In the head and neck, cicatricial pemphigoid predominantly affects the oral cavity (85–90%) and eyes (66%), but rarely affects the larynx (10–20%) and nose (15–20%) 3 4…”

“…While isolated oral cicatricial pemphigoid carries a good prognosis, disease at other sites or in multiple sites carries a worse prognosis and is typically difficult to treat 4. Examination reveals vesiculo-bullous lesions with mucous membrane scarring and involution.…”

Answer

“…There is a preponderance of females and the sixth decade 2. Complications include ocular lesions (75% of patients) with blindness in up to 20%,3 oesophageal strictures, laryngeal stenoses (8%)4 and bronchial stenoses. Flow-volume loops may show airflow disruption.…”

“…Flow-volume loops may show airflow disruption. Ocular, laryngeal, oesophageal, nasopharyngeal and genital involvement are deemed high risk for progression carrying a worse prognosis,3 the anti-epigrilin (laminin 5) variant particularly being associated with malignancy.…”