A study of desmoglein 1 autoantibodies in pemphigus vulgaris: racial differences in frequency and the association with a more severe phenotype

Background Pemphigus vulgaris (PV) is characterized by pathogenic autoantib odies to desmoglein (Dsg) 3, but additional antibodies to Dsg1, the pemphig us foliaceus antigen, are detectable in some cases. Objectives To investigate the clinical significance of the presence of both Dsg 1 and 3 antibodies. Methods In 79 subjects with PV, enzyme-linked immunosorbent assays were use d to detect IgG autoantibodies reactive with the ectodomain of Dsg1 and Dsg 3. Results There was a clear association between the clinical phenotype and th e Dsg antibody profile, All subjects had Dsg3 autoantibodies and 61% had co existing Dsg1 antibodies (Dsg3+/Dsg1+). PV limited entirely to the mucosal surfaces was seen only in Dsg3+/Dsg1- patients, while additional Dsg1 antib odies (Dsg3+/Dsg1+) predicted cutaneous in addition to mucosal involvement. Although minor cutaneous involvement was observed in most Dsg3+/Dsg1- pati ents, severe cutaneous involvement was seen only in Dsg3+/Dsg1+ patients. D sg1 antibodies were detectable early in the course of disease and their app earance did not relate to the use of systemic therapy, The proportion of Ds g1+ patients was higher in those of Indian origin compared with white north ern Europeans (P < 0.05). Conclusions These data suggest that the presence of Dsg1 antibodies is pred ictive of a potentially more severe disease and that genetic factors may de termine the Dsg antibody profile.