Background:
Pemphigus vulgaris (pv) is characterized by pathogenic autoantibodies to desmoglein (dsg) 3, but additional antibodies to dsg1, the pemphigus 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 used to detect igg autoantibodies reactive with the ectodomain of dsg1 and dsg3.
Results:
There was a clear association between the clinical phenotype and the dsg antibody profile. all subjects had dsg3 autoantibodies and 61% had coexisting dsg1 antibodies (dsg3+/dsg1+). pv limited entirely to the mucosal surfaces was seen only in dsg3+/dsg1- patients, while additional dsg1 antibodies (dsg3+/dsg1+) predicted cutaneous in addition to mucosal involvement. although minor cutaneous involvement was observed in most dsg3+/dsg1- patients, severe cutaneous involvement was seen only in dsg3+/dsg1+ patients. dsg1 antibodies were detectable early in the course of disease and their appearance did not relate to the use of systemic therapy. the proportion of dsg1+ patients was higher in those of indian origin compared with white northern europeans (p < 0.05).
Conclusions:
These data suggest that the presence of dsg1 antibodies is predictive of a potentially more severe disease and that genetic factors may determine the dsg antibody profile.