title: Autoantibodies against the processed ectodomain of collagen XVII (BPAG2, BP180) define a canine homologue of linear IgA disease of humans.
abstract: Linear IgA disease (LAD) is an acquired autoimmune subepidermal blistering dermatosis that affects human children and adults. In contrast to bullous pemphigoid, in which autoantibodies recognize transmembrane type XVII collagen (BP180, BPAG2), LAD is associated with skin-fixed and circulating IgA autoantibodies that target LAD-1, the processed extracellular form of type XVII collagen. An immunologic homologue of LAD in humans was identified in two dogs according to the following criteria: 1) erosive, ulcerative, and crusted lesions seen on the face, in the oral cavity, and on the extremities, 2) dermoepidermal clefting present in the basement membrane lamina lucida without inflammation or with mild neutrophilic infiltration, 3) basement membrane-fixed IgG and/or IgA antibodies, and 4) circulating IgA and IgG autoantibodies that target the 120-kd soluble protein LAD-1. The present study establishes unequivocally the existence of a naturally occurring canine model of LAD of humans.
authors.affiliation: Department of Clinical Sciences, College of Veterinary Medicine, North Carolina State University, Raleigh 27606, USA. thierry_olivry@ncsu.edu
authors.forename: T
authors.initials: T
authors.lastname: Olivry
authors.forename: S M
authors.initials: SM
authors.lastname: Dunston
authors.forename: M
authors.initials: M
authors.lastname: Fahey
authors.forename: N
authors.initials: N
authors.lastname: Nguyen
authors.forename: M P
authors.initials: MP
authors.lastname: Marinkovich
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000032:analysis*; D001324:Autoantigens / Q000276:immunology*; D002352:Carrier Proteins; D002460:Cell Line; D003094:Collagen / Q000276:immunology*; D003598:Cytoskeletal Proteins; D004283:Dog Diseases / Q000276:immunology*; D004285:Dogs; D000071821:Dystonin; D019085:Fluorescent Antibody Technique, Direct / Q000662:veterinary; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D012872:Skin Diseases, Vesiculobullous / Q000276:immunology / Q000662:veterinary*; D000097805:Collagen Type XVII
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D003094:Collagen; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C486268:DST protein, human