%0 Journal Article
%T Clinical Relevance of Autoantibodies in Patients with Autoimmune Bullous Dermatosis
%A Lilla Mih¨¢lyi
%A M¨¢ria Kiss
%A Attila Dobozy
%A Lajos Kem¨¦ny
%A S¨¢ndor Husz
%J Journal of Immunology Research
%D 2012
%R 10.1155/2012/369546
%X The authors present their experience related to the diagnosis, treatment, and followup of 431 patients with bullous pemphigoid, 14 patients with juvenile bullous pemphigoid, and 273 patients with pemphigus. The detection of autoantibodies plays an outstanding role in the diagnosis and differential diagnosis. Paraneoplastic pemphigoid is suggested to be a distinct entity from the group of bullous pemphigoid in view of the linear C3 deposits along the basement membrane of the perilesional skin and the ¡°ladder¡± configuration of autoantibodies demonstrated by western blot analysis. It is proposed that IgA pemphigoid should be differentiated from the linear IgA dermatoses. Immunosuppressive therapy is recommended in which the maintenance dose of corticosteroid is administered every second day, thereby reducing the side effects of the corticosteroids. Following the detection of IgA antibodies (IgA pemphigoid, linear IgA bullous dermatosis, and IgA pemphigus), diamino diphenyl sulfone (dapsone) therapy is preferred alone or in combination. The clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis is stressed. 1. Introduction The most frequent autoimmune bullous skin disorders are bullous pemphigoid (BP) and pemphigus vulgaris (PV). The diagnosis of both diseases relies not only on the clinical features but also on the detection of skin- or membrane-bound and circulating autoantibodies. We first diagnosed subepidermal bullous dermatosis in 1970 [1] by means of a direct immunofluorescence technique (DIF). We have subsequently examined, diagnosed, treated, and followed up several hundred patients with bullous skin diseases, and in this paper we present our experience in comparison with the literature findings. 2. Patients and Methods Since 1970, we have diagnosed and treated 431 patients with BP (age range 38¨C102 years, mean 71.6 years), 14 children with juvenile BP (age range 3¨C14 years, mean 7.5 years), and 273 patients with pemphigus (age range 21¨C83 years, mean 53.9 years). All clinical investigations were conducted with the understanding and the consent of the patients. We are currently treating 47 patients with pemphigus and 45 with BP. The diagnoses were based on the clinical features and routine histological and immunohistological examinations [2]. For DIF tests, we used the intact skin adjacent to the bulla as substrate and antihuman IgG, IgA, IgM, and C3 conjugates labeled with FITC for antibody detection. For indirect immunofluorescence (IIF) examinations, we used esophagus samples from monkey and rabbit, and normal human
%U http://www.hindawi.com/journals/jir/2012/369546/