SUMMARY
Objective
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.
Study Design
A retrospective cohort study.
Methods
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.
Results
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.
Conclusions
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.
Key Words
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Article info
Publication history
Published online: February 12, 2021
Accepted:
January 7,
2021
Footnotes
The authors have no funding, financial relationships, or conflicts of interest to disclose.
Identification
Copyright
© 2021 The Voice Foundation. Published by Elsevier Inc. All rights reserved.
