Ocular cicatricial pemphigoid with granular IgA and complement deposition. 1985

A D Proia, and G N Foulks, and F P Sanfilippo

A 65-year-old woman developed chronic redness of both eyes, and, over the ensuing 2 1/2 years, she had progressive conjunctival scarring with symblepharon formation. Other mucosal surfaces were not involved. A conjunctival biopsy specimen 12 months following onset of her disease showed areas of epithelial separation from the basement membrane zone as well as subepithelial chronic inflammation and scarring. Two years later, another conjunctival biopsy specimen showed granular deposition of IgA and C3 along the epithelial basement membrane zone using direct immunofluorescent staining. Electron microscopy confirmed the presence of deposits that were morphologically consistent with antigen-antibody complexes. These findings suggest that antigen-antibody (IgA) immune-complex deposition may provide an alternative pathogenetic mechanism to basement membrane zone autoantibody formation for development of progressive conjunctival scarring.

UI MeSH Term Description Entries
D007070 Immunoglobulin A Represents 15-20% of the human serum immunoglobulins, mostly as the 4-chain polymer in humans or dimer in other mammals. Secretory IgA (IMMUNOGLOBULIN A, SECRETORY) is the main immunoglobulin in secretions. IgA,IgA Antibody,IgA1,IgA2,Antibody, IgA
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
D003228 Conjunctiva The mucous membrane that covers the posterior surface of the eyelids and the anterior pericorneal surface of the eyeball. Bulbar Conjunctiva,Palpebral Conjunctiva,Plica Semilunaris of Conjunctiva,Plicae Semilunares of Conjunctiva,Tunica Conjunctiva,Conjunctiva, Bulbar,Conjunctiva, Palpebral,Conjunctivas
D003229 Conjunctival Diseases Diseases involving the CONJUNCTIVA. Conjunctival Disease,Disease, Conjunctival,Diseases, Conjunctival
D005260 Female Females
D005455 Fluorescent Antibody Technique Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy. Antinuclear Antibody Test, Fluorescent,Coon's Technique,Fluorescent Antinuclear Antibody Test,Fluorescent Protein Tracing,Immunofluorescence Technique,Coon's Technic,Fluorescent Antibody Technic,Immunofluorescence,Immunofluorescence Technic,Antibody Technic, Fluorescent,Antibody Technics, Fluorescent,Antibody Technique, Fluorescent,Antibody Techniques, Fluorescent,Coon Technic,Coon Technique,Coons Technic,Coons Technique,Fluorescent Antibody Technics,Fluorescent Antibody Techniques,Fluorescent Protein Tracings,Immunofluorescence Technics,Immunofluorescence Techniques,Protein Tracing, Fluorescent,Protein Tracings, Fluorescent,Technic, Coon's,Technic, Fluorescent Antibody,Technic, Immunofluorescence,Technics, Fluorescent Antibody,Technics, Immunofluorescence,Technique, Coon's,Technique, Fluorescent Antibody,Technique, Immunofluorescence,Techniques, Fluorescent Antibody,Techniques, Immunofluorescence,Tracing, Fluorescent Protein,Tracings, Fluorescent Protein
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000267 Tissue Adhesions Pathological processes consisting of the union of the opposing surfaces of a wound. Adhesions, Tissue,Surgery-Induced Tissue Adhesions,Surgical Adhesions,Adhesion, Surgery-Induced Tissue,Adhesion, Surgical,Adhesion, Tissue,Adhesions, Surgery-Induced Tissue,Adhesions, Surgical,Surgery Induced Tissue Adhesions,Surgery-Induced Tissue Adhesion,Surgical Adhesion,Tissue Adhesion,Tissue Adhesion, Surgery-Induced,Tissue Adhesions, Surgery-Induced

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