Mucous membrane pemphigoid–otorhinolaryngological manifestations: a retrospective cohort study

Introduction: Mucous membrane pemphigoid (MMP) refers to a heterogeneous group of rare, chronic, inflammatory, mucous membrane-dominated, sub-epithelial blistering diseases that manifest with a varying constellation of oral, ocular, cutaneous, genital, nasopharyngeal, esophageal, and laryngeal lesions. MMP can progress to scarring in affected areas, which may lead to devastating complications including ocular involvement leading to blindness as well as laryngeal involvement leading to airway obstruction. Materials and methods: A retrospective chart review was conducted for patients that were followed in two tertiary academic centers between 2009 through 2017 for upper aerodigestive tract manifestations of MMP. Patients with significant underlying skin involvement suggestive of bullous pemphigoid as well as MMP with isolated ocular involvement were excluded. Results: Twenty-seven patients were diagnosed with MMP and followed in two tertiary referral medical centers. The most common site of initial presentation was the oral cavity, and all patients had oral cavity involvement at some point. Two-thirds of our patients had complete remission or remission with intermittent relapses disease. Patients with mild to moderate disease showed excellent response to topical steroid treatment. For more severe disease, high-dose prednisone was insufficient, as in most cases relapse occurred at some point of time during tapering down. Conclusions: MMP is a rare autoimmune disorder that may present with a wide spectrum of head and neck manifestations and, potentially, catastrophic sequelae. This work highlights the experience of two tertiary centers with MMP in an attempt to draw attention to this unusual disorder.