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Anti-Epiligrin Cicatricial Pemphigoid With Antibodies Against the  2 Subunit of Laminin 5
Hossein C. Nousari, MD;
Adrienne Rencic, MD, PhD;
Roger Hsu, MD;
Kim B. Yancey, MD;
Grant J. Anhalt, MD
Arch Dermatol. 1999;135:173-176.
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 infrequently 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  3 and the  2 subunit of laminin 5.
Conclusion This article expands the diversity of the clinical and immunopathologic features of this newly characterized variant of CP.
From the Department of Dermatology, Johns Hopkins Medical Institutions, Baltimore (Drs Nousari, Rencic, Hsu, and Anhalt) and the Dermatology Branch, National Cancer Institute, National Institutes of Health, Bethesda (Dr Yancey), Md.
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