Abstract
Abstract Pemphigus foliaceus (PF) and pemphigus vulgaris (PV) are autoimmune skin blistering diseases characterized by pathogenic autoantibodies against desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3), respectively. Fogo selvagem (FS) is the endemic form of PF. To study the etiology of PV and FS, hybridomas were generated from the B cells of 5 PV patients and 7 FS patients. The V genes of both H and L chain of the Dsg-specific autoantibodies from these hybridomas were sequenced and analyzed for the H and L chain pairing of the autoantibodies, the diversity of H and L chain V gene usage, and the extent of somatic mutation. The number of somatic mutations in the expressed genes ranged from zero to 42, and the replacement to silent mutation (R/S) ratio was much higher in CDRs than in FWRs of both IgM and IgG hybridomas from FS patients. The R/S ratio was also much higher in CDRs than in FWRs of IgG hybridomas from PV patients. In addition, clonally related sets of anti-Dsg1 or anti-Dsg3 hybridomas were identified from individual FS or PV patients based on VH-D-JH junctional sequence identity that reveals ongoing somatic mutation during clonal expansion. Thus, our evidence suggests that the anti-Dsg autoimmune response in both PV and FS is antigen selected.
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