title: Ocular and oral mucous membrane pemphigoid (cicatricial pemphigoid).
abstract: Mucous membrane pemphigoid, a heterogeneous group of autoimmune blistering diseases, affects primarily the mucous membranes. Although oral and ocular mucosae can both be affected in a given patient, patients with involvement restricted to oral mucosae tend to have a benign outcome, whereas those with ocular disease commonly face treatment resistance, resulting in scarring and blindness. Diagnosis requires direct immunofluorescence microscopy to demonstrate a linear deposition of immunoglobulin (Ig) G or IgA, or complement component 3 (C3), at the epithelial basement membrane. Although the target antigens vary, subsets of patients affected exclusively by oral and ocular mucosal diseases have autoantibodies targeting α-6 and β-4 integrins, respectively.
authors.affiliation: Department of Dermatology, University of Illinois College of Medicine, Chicago, USA. larrycha@uic.edu
authors.forename: Lawrence S
authors.initials: LS
authors.lastname: Chan
mesh_terms: D001485:Basement Membrane / Q000276:immunology / Q000473:pathology; D003176:Complement C3 / Q000032:analysis; D003228:Conjunctiva / Q000276:immunology* / Q000473:pathology*; D005882:Gingival Diseases / Q000276:immunology* / Q000473:pathology*; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D008856:Microscopy, Fluorescence; D009061:Mouth Mucosa / Q000276:immunology / Q000473:pathology; D009092:Mucous Membrane / Q000382:microbiology / Q000473:pathology; D010390:Pemphigoid, Benign Mucous Membrane / Q000276:immunology* / Q000473:pathology*
chemical_list: D003176:Complement C3; D007070:Immunoglobulin A; D007074:Immunoglobulin G