Anti-epiligrin cicatricial pemphigoid with antibodies against the γ2 subunit of laminin 5

Hossein C. Nousari, Adrienne Rencic, Roger Hsu, Kim B. Yancey, Grant J. Anhalt

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish (US)
Pages (from-to)173-176
Number of pages4
JournalArchives of dermatology
Volume135
Issue number2
DOIs
StatePublished - Feb 1999

ASJC Scopus subject areas

  • Dermatology

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