Background: Cicatricial pemphigoid (CP) is a scarring subepithelial mucocutaneous blistering disease characterized by anti-basement membrane zone autoantibodies. Anti-epiligrin CP is an uncommon variant that has been recently characterized. Severe laryngeal involvement is in, frequently observed in all forms of CP and has been documented in only 2 patients with anti-epiligrin CP. Observations: We report a case of CP exhibiting extensive laryngeal and ocular involvement. Histological, immunofluorescence, and immunoprecipitation studies confirmed the diagnosis of anti-epiligrin CP. Immunoblotting studies demonstrated the presence of antibodies against the α3 and the γ2 subunit of laminin 5. Conclusion: This article expands the diversity of the clinical and immunopathologic features of this newly characterized variant of CP.
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