Biomaterial Database

Antiepiligrin cicatricial pemphigoid. 2000

R C Hsu, and Z Lazarova, and H G Lee, and Y C Tung, and H S Yu

Department of Dermatology, Kaohsiung Medical College,Taiwan.

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.

UI	MeSH Term	Description	Entries
D007074	Immunoglobulin G	The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.	Gamma Globulin, 7S,IgG,IgG Antibody,Allerglobuline,IgG(T),IgG1,IgG2,IgG2A,IgG2B,IgG3,IgG4,Immunoglobulin GT,Polyglobin,7S Gamma Globulin,Antibody, IgG,GT, Immunoglobulin
D007797	Laminin	Large, noncollagenous glycoprotein with antigenic properties. It is localized in the basement membrane lamina lucida and functions to bind epithelial cells to the basement membrane. Evidence suggests that the protein plays a role in tumor invasion.	Merosin,Glycoprotein GP-2,Laminin M,Laminin M Chain,Chain, Laminin M,Glycoprotein GP 2,M Chain, Laminin
D008297	Male		Males
D010390	Pemphigoid, Benign Mucous Membrane	A chronic blistering disease with predilection for mucous membranes and less frequently the skin, and with a tendency to scarring. It is sometimes called ocular pemphigoid because of conjunctival mucous membrane involvement.	Pemphigoid, Cicatricial,Benign Mucosal Pemphigoid,Benign Mucous Membrane Pemphigoid,Cicatricial Pemphigoid, Ocular,Mucous Membrane Pemphigoid, Benign,Ocular Cicatricial Pemphigoid,Pemphigoid, Ocular Cicatricial,Benign Mucosal Pemphigoids,Cicatricial Pemphigoid,Cicatricial Pemphigoids, Ocular,Mucosal Pemphigoid, Benign,Mucosal Pemphigoids, Benign,Ocular Cicatricial Pemphigoids,Pemphigoid, Benign Mucosal,Pemphigoids, Benign Mucosal,Pemphigoids, Ocular Cicatricial
D003176	Complement C3	A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.	C3 Complement,C3 Precursor,Complement 3,Complement C3 Precursor,Complement Component 3,Precursor-Complement 3,Pro-C3,Pro-Complement 3,C3 Precursor, Complement,C3, Complement,Complement, C3,Component 3, Complement,Precursor Complement 3,Precursor, C3,Precursor, Complement C3,Pro C3,Pro Complement 3
D004817	Epidermis	The external, nonvascular layer of the skin. It is made up, from within outward, of five layers of EPITHELIUM: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis).
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000097794	Kalinin	An epithelium-specific basement membrane adhesion molecule that is a component of anchoring filaments. Consists of 3 subunits (A, B2, & B1).	Epiligrin,Kalinin B1,Laminin 332,Laminin 5,Laminin B1k,Laminin-5,Laminin-5 beta3 Protein,Nicein,BM 600,BM-600,LAMB3 protein
D000368	Aged	A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available.	Elderly
D001323	Autoantibodies	Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.	Autoantibody