Biomaterial Database

Bullous and cicatricial pemphigoid. 1991

G J Anhalt, and L H Morrison

Johns Hopkins University, Department of Dermatology, Baltimore, Maryland 21205.

Bullous pemphigoid (BP) and cicatricial pemphigoid are blistering mucocutaneous diseases characterized by detachment of the overlying epithelium from its stroma. IgG and complement components are deposited in all affected tissue at the level of blister formation--through the lamina lucida of the epithelium. The primary antibody response is of the IgG 4 subclass, and the antigens recognized by these autoantibodies have been shown to be 230 kD and 180 kD transmembrane proteins unique to the hemidesmosome of stratified squamous epithelial cells. Although it is suspected that these antigens are important in cell-substrate adhesion, this has not been proven. Stanley et al. have recently defined the sequence of a portion of the C terminal end of the 230 kD antigen and Diaz et al. have isolated a cDNA encoding for the 180 kD antigen. Structural data regarding these antigens should prove critical to definition of their presumed function. Therefore, BP is felt to be an autoimmune disease where the cutaneous lesions may solely be a consequence of binding of these antihemidesmosome autoantibodies to the specific antigen, but definitive proof of this assumption is incomplete.

UI	MeSH Term	Description	Entries
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
D010391	Pemphigoid, Bullous	A chronic and relatively benign subepidermal blistering disease usually of the elderly and without histopathologic acantholysis.	Pemphigoid,Bullous Pemphigoid,Pemphigoids
D003165	Complement System Proteins	Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).	Complement Proteins,Complement,Complement Protein,Hemolytic Complement,Complement, Hemolytic,Protein, Complement,Proteins, Complement,Proteins, Complement System
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001323	Autoantibodies	Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.	Autoantibody
D001324	Autoantigens	Endogenous tissue constituents with the ability to interact with AUTOANTIBODIES and cause an immune response.	Autoantigen,Autologous Antigen,Autologous Antigens,Self-Antigen,Self-Antigens,Antigen, Autologous,Antigens, Autologous,Self Antigen,Self Antigens
D001485	Basement Membrane	A darkly stained mat-like EXTRACELLULAR MATRIX (ECM) that separates cell layers, such as EPITHELIUM from ENDOTHELIUM or a layer of CONNECTIVE TISSUE. The ECM layer that supports an overlying EPITHELIUM or ENDOTHELIUM is called basal lamina. Basement membrane (BM) can be formed by the fusion of either two adjacent basal laminae or a basal lamina with an adjacent reticular lamina of connective tissue. BM, composed mainly of TYPE IV COLLAGEN; glycoprotein LAMININ; and PROTEOGLYCAN, provides barriers as well as channels between interacting cell layers.	Basal Lamina,Basement Lamina,Lamina Densa,Lamina Lucida,Lamina Reticularis,Basement Membranes,Densas, Lamina,Lamina, Basal,Lamina, Basement,Lucida, Lamina,Membrane, Basement,Membranes, Basement,Reticularis, Lamina