Research On The Phenomenon That The Clinical Phenotype Of Pemphigus Does Not Correspond To The Antibody Profile

Objective: To sort out the data of pemphigus patients whose anti-Dsg1/3 IgG is not consistent with the clinical phenotype according to the compensation theory of desmoglein,to analyze and confirm the reliability of the results,and to collate and report a phenomenon that challenges the previous theory.Methods: The clinical data and peripheral blood samples of patients whose antibody spectrum did not correspond to the clinical phenotype admitted to the Wuhan NO.1 Hospital from 2017 to 2018 were collected.The serum of patients with pemphigus was tested by immunoblotting.The results were compared with the ELISA and the presence of other antigens was observed.Results: 1.Five cases with clinical phenotypes that did not correspond to the antibody spectrum and three cases with clinical phenotypic transformation during the course of disease were sorted out and summarized into four clinically rare phenomena;2.The target protein(130kDa,160kDa)was not detected in the experiment.Only a small amount of protein fragments were detected.The 10 kDa fragment was only positive in the serum of patients with pemphigus,but negative in the control group.Conclusion:1.Phenomenon one: Anti-Dsg3 IgG positive,but without mucosal damage,with or without skin damage,consider the presence of weak pathogenic anti-Dsg3 antibody in serum;Phenomenon two: only anti-Dsg1 IgG positive,without skin and mucous membrane damage,no literature support,the meaning is unknown;phenomenon three: only anti-Dsg1 IgG positive,but only mucosal damage,may be related to the presence of other non-Dsg antigens;phenomenon four: some pemphigus vulgaris The clinical phenotype may be shifted during follow-up,possibly due to epitope spreading phenomenon.2.Using normal skin extract as an antigen substrate for immunoblotting experiments to detect pemphigus antigen is correct,but the experimental process is complicated,and considering that the surface protein of keratinocytes is easily degraded after processing,it is not easy to have a positive result;the pathogenesis of pemphigus is a very complex process involving many structures that regulate the adhesion and survival of keratinocytes,autoantibodies to related proteins,and the like.Therefore,we still need to do a lot of work to determine the pathogenicity of these autoantibodies and provide a basis for the formation of new theories.