Laryngeal Pemphigoid Evolution and Response to Treatment

Area of Science:

• Otolaryngology
• Dermatology
• Immunology

Background:

• Laryngeal pemphigoid (LP) is a rare autoimmune blistering disease affecting the larynx.
• Understanding LP's clinical course and treatment response is crucial for patient management.

Purpose of the Study:

• To describe the experience in treating laryngeal pemphigoid patients.
• To identify predictors of treatment response in laryngeal pemphigoid.

Main Methods:

• Retrospective cohort study of laryngeal pemphigoid patients from March 2013 to August 2020.
• Analysis of demographics, disease characteristics, comorbidities, and serological profiles.
• Exploration of relationships between disease severity, treatment response, and diagnostic markers.

Main Results:

• Eight patients (7 female, 1 male, mean age 79) with mucous membrane pemphigoid or bullous pemphigoid were studied.
• Two patients achieved complete laryngeal remission, four partial, and two no remission.
• Absence of IgG auto-antibodies correlated with topical corticosteroid response; presence of BP180-C-terminal IgG auto-antibodies indicated resistant, progressive disease.

Conclusions:

• Laryngeal pemphigoid exhibits variable severity and treatment resistance.
• Absence of IgG auto-antibodies may predict response to topical corticosteroids.
• Presence of IgG auto-antibodies targeting BP180-C-terminal domain suggests severe scarring potential, necessitating early advanced treatment.