Anti-epiligrin cicatricial pemphigoid with antibodies against the gamma 2 subunit of laminin 5

Background: Cicatricial pemphigoid (CP) is a scarring subepithelial mucocut aneous blistering disease characterized by anti-basement membrane zone auto antibodies. Anti-epiligrin CP is an uncommon variant that has been recently characterized. Severe laryngeal involvement is infrequently observed in al l forms of CP and has been documented in only 2 patients with anti-epiligri n CP. Observations: We report a case of CP exhibiting extensive laryngeal and ocu lar involvement. Histological, immunofluorescence, and immunoprecipitation studies confirmed the diagnosis of anti-epiligrin CP. Immunoblotting studie s demonstrated the presence of antibodies against the alpha 3 and the gamma 2 subunit of laminin 5. Conclusion: This article expands the diversity of the clinical and immunopa thologic features of this newly characterized variant of CP.