Pemphigus herpetiformis with IgA and IgG antibodies to desmoglein 1 and IgG antibodies to desmocollin 3.

We describe a case with clinical and histologic features of pemphigus herpetiformis associated with IgG and IgA anti-keratinocyte cell surface antibodies to desmoglein 1 (Dsg1) and exclusively IgG antibodies to desmocollin 3 (Dsc3). The clinical presentation was somewhat similar to IgA pemphigus; the main difference was the prevailing association with IgG antibodies to Dsg1. The presence of IgA anti-Dsg1 antibodies was confirmed by IgA enzyme-linked immunosorbent assay and IgG anti-Dsc3 antibodies were detected by a novel enzyme-linked immunosorbent assay with the use of baculovirus expressing recombinant Dscs for IgG and IgA. The reactivity of IgG antibodies with Dsc3 was confirmed by COS-7 cell cDNA transfection method using cDNA of human Dsc1 to Dsc3. We discuss the differentiation of pemphigus herpetiformis, associated with both IgG and IgA antibodies, from IgA pemphigus, particularly in regard to the autoimmune reaction with Dsgs and Dscs.