Abstract Body

ABSTRACT

Background: Mucous membrane pemphigoid is an autoimmune subepidermal/submucosal disease, characterized by blisters, erosions. Its unique feature is that as the blister heal they cause irreversible scarring, which may be fatal or significantly affect the quality of life. While upper airway involvement is well studied, lower airway involvement has not been characterized. This review describes the clinical presentation, diagnosis, and treatment of pulmonary pemphigoid, present in the literature.

OBSERVATIONS: We searched Pubmed, Embase, Medline, and Google Scholar databases from inception to October 2021. 11 original case reports describing mucous membrane pemphigoid patients with biopsy and immunopathology proven tracheal and bronchial involvement were included in this review. Among 11 patients with mucous membrane pemphigoid of the lower airways, 80% were under 40 years of age. All had severe widespread mucous membrane pemphigoid involving three or five mucosal tissues. Involvement of oral cavity occurred in 100% of cases. Ocular and cutaneous involvements were seen in 82% of patients. Pulmonary involvement occurred at 24 mo (range 2-372) after the onset of mucous membrane pemphigoid. Bronchoscopy revealed acute inflammation during active disease and scarring of the trachea and bronchi in the later stage. Systemic infections occurred in 45% of the patients while the pulmonary infection occurred in 36%. Mortality due to respiratory failure at the median age of 20 years (range 18-76), occurred in 45% of the patients.

CONCLUSIONS: The young age, similarity in clinical profile, clinical course, response to systemic therapy, and high mortality warrant early recognition of pulmonary pemphigoid. These patients differ from other mucous membranous patients. Thus far it is a less recognized entity. The diagnosis could have been possibly missed in many cases. The cause of death appears to be disease related. Early diagnosis with appropriate management could produce better clinical outcomes and prevent mortality.