Abstract
BackgroundMucocutaneous blistering is characteristic of autoimmune bullous dermatoses (AIBD). Blisters are caused by autoantibodies directed against structural components of the skin. Hence, detection of specific autoantibodies has become a hallmark for AIBD diagnosis. Studies on prevalence of AIBD autoantibodies in healthy individuals yielded contradictory results.MethodsTo clarify this, samples from 7063 blood donors were tested for presence of anti-BP180-NC16A, anti-BP230 and anti-Dsg1/3 IgG by indirect immunofluorescence (IF) microscopy using a biochip.ResultsCumulative prevalence of these autoantibodies was 0.9 % (CI: 0.7–1.1 %), with anti-BP180-NC16A IgG being most prevalent. Validation of IF findings using ELISA confirmed presence of autoantibodies in 7/15 (anti-Dsg1), 6/7 (anti-Dsg3), 35/37 (anti-BP180-NC16A) and 2/3 (anti-BP230) cases. Moreover, in 16 samples, anti-BP180-NC16A autoantibody concentrations exceeded the cut-off for the diagnosis of bullous pemphigoid. Interestingly, these anti-BP180-NC16A autoantibodies from healthy individuals formed immune complexes with recombinant antigen and dose-dependently activated neutrophils in vitro. However, fine-epitope mapping within NC16A showed a different binding pattern of anti-BP180-NC16A autoantibodies from healthy individuals compared to bullous pemphigoid patients, while IgG subclasses were identical.ConclusionsCollectively, we here report a low prevalence of AIBD autoantibodies in a large cohort of healthy individuals. Furthermore, functional analysis shows differences between autoantibodies from healthy donors and AIBD patients.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-015-0278-x) contains supplementary material, which is available to authorized users.
Highlights
Mucocutaneous blistering is characteristic of autoimmune bullous dermatoses (AIBD)
Depending on the location of the blister and the targeted autoantigens, AIBD can be classified as pemphigus and pemphigoid disease, epidermolysis bullosa acquisita (EBA) and dermatitis herpetiformis [7, 8]
Clinically healthy individuals have not been systematically investigated for the presence of autoantibodies to structural proteins of the skin and the reported autoantibody prevalence is contradictory
Summary
Mucocutaneous blistering is characteristic of autoimmune bullous dermatoses (AIBD). Blisters are caused by autoantibodies directed against structural components of the skin. Studies analyzed serum samples obtained from individuals before they received a diagnosis of systemic lupus erythematousus (SLE) or rheumatoid arthritis (RA). These studies clearly demonstrated the presence of autoantibodies several years before diagnosis [17, 18]. Derived from these findings, one may assume that autoantibodies in AIBD predate the onset of the corresponding disease. Clinically healthy individuals have not been systematically investigated for the presence of autoantibodies to structural proteins of the skin and the reported autoantibody prevalence is contradictory. The potential pathogenic relevance of the detected autoantibodies was evaluated
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
