Relationship between clinical findings of patients with bullous pemphigoid and antigens recognized by their circulating antibasement membrane zone antibodies

• Published in: Annales de dermatologie et de vénéréologie 2004-04, Vol.131 (4), p.333-337
• Main Authors: Gary, A, Carvalho, P, Louison, J-B, Helot, M-F, Gilbert, D, Bernard, P, Roujeau, J-C, Bedane, C, Delaporte, E, Vaillant, L, Dreno, B, Saiag, P, Tancrede-Bohin, E, Plantin, P, D'Incan, M, Sassolas, B, Lok, C, Labeille, B, Pauwels, C, Chosidow, O, Picard, C, Loche, F, Guillaume, J-C, Joly, P
• Format: Article
• Language: fre
• Subjects: Aged; Aged, 80 and over; Autoantibodies - blood; Autoantigens - blood; Basement Membrane - immunology; Female; Humans; Male; Pemphigoid, Bullous - blood; Pemphigoid, Bullous - immunology; Prospective Studies
• ISSN: 0151-9638
• DOI: 10.1016/S0151-9638(04)93611-3

Clinical features and extent of bullous pemphigoid lesions differed widely among patients. The pathogenic role of anti-BPAG2 antibodies has been recently demonstrated. The aim of this study was to analyze the relationship between clinical features of bullous pemphigoid patients and the antigens recognized by their serum. One hundred and twelve bullous pemphigoid patients were included in this prospective multicenter study. Inclusion criteria were the following: 1) diagnosis of bullous pemphigoid established on the presence of 3 of the 4 clinical features of bullous pemphigoid, histological picture of bullous pemphigoid and positive direct immunofluorescence; 2) serum available for immunoblotting studies. The clinical and biological findings were prospectively recorded on standard forms. Sera were collected and analyzed using indirect immunofluorescence and immunoblotting on human epidermal extracts. Analysis of patient's clinical features depending on the antigens recognized by their serum showed that patients whose serum contained anti-BPAG1 antibodies had more frequently pruritus, blisters on the lower limbs and a positive indirect immunofluorescence. Patients whose serum contained anti-BPAG2 antibodies had blisters more frequently localized on the head, and a more frequently negative indirect immunofluorescence. Patients whose serum was negative by immunoblotting had less frequently urticarial and/or eczematous lesions, bullae less frequently localized on the lower part of the trunk, abdomen and lower limbs, lower eosinophilia and a more frequently negative indirect immunofluorescence. Patients with circulating anti-BPAG1 antibodies exhibited the most typical, clinical and biological features of bullous pemphigoid.