Mucous membrane pemphigoid involving the upper respiratory tract: A case series and literature review

Rationale: We present a case series involving 4 patients diagnosed with upper respiratory tract mucous membrane pemphigoid (MMP) at a tertiary referral center, accompanied by a comprehensive literature review. Patient concerns: This study focuses on cases of MMP that presented with respiratory symptoms and were treated at the Department of Otolaryngology-Head and Neck Surgery, West China Hospital, from 2018 to 2023. In addition, it reviews cases of MMP affecting the upper respiratory tract that have been published on PubMed from 2013 to 2023. Diagnoses: Four patients were diagnosed with MMP affecting the upper respiratory tract. Interventions: One patient required a tracheotomy due to severe dyspnea, while the other 3 initially presented with symptoms affecting various regions of the upper respiratory tract. Diagnosis for 3 of the patients was confirmed through direct immunofluorescence of skin or mucosal samples, in conjunction with pathological examination, and they were subsequently treated with systemic glucocorticoids. Outcomes: Notably, the second patient exhibited favorable outcomes when glucocorticoids were combined with methotrexate. The third patient’s condition showed gradual improvement following the initial glucocorticoid treatment, and intravenous immunoglobulin and rituximab were later administered to alleviate symptoms. Following the tracheotomy, the fourth patient’s disease was effectively managed with regular corticosteroid treatment in the Dermatology Department. Lessons: Considering the prevalent symptoms associated with upper respiratory tract MMP and the severe repercussions of failing to intervene, early diagnosis and the timely administration of systemic corticosteroids are of paramount importance.