ANCA associated with Behçet's disease.
Behçet's disease is a multisystem disorder affecting primarily mucocutaneous and ocular sites although the gastrointestinal, cardiovascular, central nervous and respiratory systems may also be involved. Hulusi Behçet, a Turkish dermatologist, first described Behçet's disease in 1937 and suggested a possible infectious aetiology. The pathogenesis of this condition still remains unclear although it is likely that infection acts as a trigger in genetically susceptible individuals. We report a patient with unusual cutaneous manifestations of Behçet's disease and antineutrophil cytoplasmic antibodies (ANCA) directed against the cytotoxic protein, bactericidal/permeability-increasing protein (BPI). This is the first report of Behçet's disease associated with this autoantibody.
Burrows
N P
NP
Department of Dermatology, Addenbrooke's Hospital Cambridge, England.
Zhao
M H
MH
Norris
P G
PG
Lockwood
C M
CM
D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies / Q000032:analysis*; D001327:Autoimmune Diseases / Q000276:immunology*; D001528:Behcet Syndrome / Q000150:complications / Q000276:immunology*; D006801:Humans; D008297:Male; D017445:Skin Diseases, Vascular / Q000209:etiology; D014657:Vasculitis / Q000209:etiology
D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies