Laryngeal Pemphigoid Evolution and Response to Treatment

Abstract

This study aimed to present our experience in treating laryngeal pemphigoid (LP) patients, including disease course, treatment and treatment response, and to search for predictors of response to treatment. A retrospective cohort study. The medical records of all patients with LP from March 2013 to August 2020 were reviewed. Potential relationships between disease severity and response to treatment and demographics, diagnosis, extent of laryngeal and extra-laryngeal involvement, comorbidities, immunostaining, and serology profile were explored. Eight patients were included in the study (seven females, one male, mean age 79 years, mean follow-up 22 months). Diagnoses included mucous membrane pemphigoid (MMP, n=5), bullous pemphigoid (BP, n=3). Two patients achieved complete laryngeal remission, four achieved partial remission, and two had no remission. The time to achieve laryngeal disease control was longer than for extra-laryngeal disease (P = 0.02). Potential associations were found between the absence of immunoglobulin G (IgG)-type auto-antibodies deposits in the basement membrane zone and a laryngeal disease that responded to topical corticosteroids and between the presence of BP180-C-terminal IgG auto-antibodies and a resistant rapidly progressive laryngeal disease. LP has a spectrum of severity, variable response and is more resistant to treatment. The absence of IgG-type auto-antibodies may indicate a response to topical corticosteroids. Based on our limited observation, the presence of IgG-type auto-antibodies that target BP180-C-terminal domain may indicate a more severe scarring disease. Early recognition of these "high-risk patients" will allow early initiation of advanced systemic treatment that may prevent the irreversible effects of scarring.