Anti-epiligrin cicatricial pemphigoid with antibodies against the gamma2 subunit of laminin 5.
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.


METHODS
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 alpha3 and the gamma2 subunit of laminin 5.


CONCLUSIONS
This article expands the diversity of the clinical and immunopathologic features of this newly characterized variant of CP.
Department of Dermatology, Johns Hopkins Medical Institutions, Baltimore, MD 21205, USA.
H C
HC
Nousari
A
A
Rencic
R
R
Hsu
K B
KB
Yancey
G J
GJ
Anhalt
D000368:Aged; D000906:Antibodies / Q000097:blood*; D015815:Cell Adhesion Molecules / Q000276:immunology*; D006801:Humans; D007797:Laminin / Q000276:immunology*; D008297:Male; D010390:Pemphigoid, Benign Mucous Membrane / Q000276:immunology*; D000097794:Kalinin
D000906:Antibodies; D015815:Cell Adhesion Molecules; D007797:Laminin; D000097794:Kalinin