Abstract
Pemphigus is clinically divided into three subtypes : pemphigus vulgaris with a dominant mucosal lesion (PV-M), mucocutaneous lesion (PV-MC), and typical pemphigus foliace (PF). Several cases have changed clinical phenotypes among the three pemphigus phenotypes during the in clinical courses. Mean while a novel enzyme-linked immunosorbent assay (ELISA) with recombinant desmoglein (rDsg1) and rDsg3 has been developed as a highly sensitive and specific diagnostic tool for pemphigus. We experienced three cases undergoing clinical transition from PV-MC to PF, eight cases from PV-MC to PV-M, and one case from PV-M to PVMC. Their sera were obtained and the anti-Dsg antoantibody titers were analysed using ELISA. The clinical changes were clearly associated with the change of autoantibody profile from Dsg3+/1+ to Dsg3–/1+, from Dsg3+/1+ to Dsg3+/1–, and from Dsg3+/1– to Dsg3+/1+, respectively, confirming that the clinical transition among the three pemphigus phenotypes correlates well with changes in the titers of either anti-Dsg1 or anti-Dsg3 autoantibodies, We conclude that it is advantage to perform the Dsg ELISA during the time course of this disease, because we can promptly and accurately catch any transformation among the pemphigus phenotypes.
