Antiepiligrin cicatricial pemphigoid. J Am Acad Dermatol 2000 May;42(5 Pt 1):841-4
Date
04/25/2000Pubmed ID
10775868DOI
10.1067/mjd.2000.104790Scopus ID
2-s2.0-0034073904 (requires institutional sign-in at Scopus site) 11 CitationsAbstract
Cicatricial pemphigoid is a chronic subepithelial autoimmune blistering disease of mucous membranes and skin. Recently, a subtype of cicatricial pemphigoid with autoantibodies to epiligrin was identified. We describe a Taiwanese patient who presented with ocular, oral, and cutaneous involvement. Direct immunofluorescence showed IgG and C3 deposition in epidermal basement membrane; indirect immunofluorescence showed circulating IgG autoantibodies reactive with the dermal side of 1 mol/L sodium chloride-split skin. Immunoblotting of laminin 5 isolated from the extracellular matrix of cultured human keratinocytes showed no specific reactivity. In contrast, with immunoprecipitation of the conditioned culture media from biosynthetically radiolabeled human keratinocytes, this patient's serum clearly reacted with a series of disulfide-linked polypeptides that correspond to laminin 5(alpha3beta3gamma2) and laminin 6(alpha3beta1gamma1). This is the first confirmed case of a patient of Chinese ancestry with this disease entity.
Author List
Hsu RC, Lazarova Z, Lee HG, Tung YC, Yu HSMESH terms used to index this publication - Major topics in bold
AgedAutoantibodies
Basement Membrane
Cell Adhesion Molecules
Complement C3
Epidermis
Humans
Immunoglobulin G
Laminin
Male
Pemphigoid, Benign Mucous Membrane
Skin
