Clinical, histopathological and immunological distinction in two cases of IgA pemphigus

Abstract

Two cases of IgA pemphigus, one of the subcorneal pustular dermatosis (SPD) type and one of the intraepidermal neutrophilic IgA dermatosis (IEN) type, are described. IgA anti-keratinocyte cell surface antibodies reacted only with the uppermost epidermis in the SPD type but with the entire epidermis in the IEN type. A cDNA transfection test for desmocollins (Dsc) revealed that IgA antibodies of the SPD type reacted with Dsc1, but the IEN type did not react with any of the Dsc isoforms (Dsc1-3). ELISA for desmoglein 1 (Dsg1) and Dsg3 showed that neither of the cases had antibodies to Dsg - of either the IgG or IgA subtype. These results confirm that the autoantigen in SPD-type IgA pemphigus is Dsc1, whereas the antigen in most cases of IEN-type IgA pemphigus is unknown.