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Autoantibodies against the Processed Ectodomain of Collagen XVII (BPAG2, BP180) Define a Canine Homologue of Linear IgA Disease of Humans

Department of Clinical Sciences, College of Veterinary Medicine, North Carolina State University, Raleigh, NC (TO, SMD); Department of Dermatology, School of Medicine, University of North Carolina, Chapel Hill, NC (TO); Dermatology Section, Palo Alto VA Health Care System (MPM); and Department of Dermatology, Stanford University, School of Medicine, Palo Alto, CA (MPM, MF, NN)

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.

Key words: Autoimmunity; BP180; BPAG2; collagen XVII; dogs; epidermal basement membrane; LAD-1; linear IgA bullous disease; skin.

Request for reprints from Dr. T. Olivry, Deparment of Clinical Sciences, College of Veterinary Medicine, North Carolina State University, 4700 Hillsborough Street, Raleigh, NC 27606 (USA). E-mail: thierry_olivry{at}ncsu.edu.

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