Changes in clinical features, histologic findings, and antigen profiles with development of pemphigus foliaceus from pemphigus vulgaris

Three Cases of Transition from Pemphigus vulgaris to Pemphigus foliaceus Confirmed by Desmoglein ELISA

“…A few cases have been reported in the literature. Earlier cases were confirmed by histology and immunoblotting [1,2,3,4,5,6] while the transition in more recent cases has been demonstrated by a change in desmoglein (Dsg) 1 and 3 autoantibody profile by Dsg ELISA testing [6,7,8,9,10,11]. We report 3 cases that showed transition from PV to PF confirmed by Dsg ELISA.…”

“…In the literature, there are at least 16 reported cases of transition of PV to PF [1,2,3,4,5,6,7,8,9,10,11] including one that reverted to PV again [7] (table 1). In contrast, there are only 3 reported cases of transition from PF to PV [1, 6, 7] and 3 cases of PV-PF overlap [4, 6].…”

“…The majority of cases of transition from PV to PF were reported by the Japanese [1,2,3,6,7,8,9]. Most of the earlier cases were confirmed on immunoblotting during the PF stage [1,2,3, 6] while in the cases reported over the past 3 years, the change in autoantibody profile has been demonstrated using Dsg ELISA [6,7,8,9,10,11]. One recent case report of transition from PV to PF documented rising Dsg1 antibody levels and decreasing Dsg3 antibody levels by ELISA with subsequent disappearance of Dsg3 antibodies while on prednisolone and immunosuppressives [11].…”

“…One recent case report of transition from PV to PF documented rising Dsg1 antibody levels and decreasing Dsg3 antibody levels by ELISA with subsequent disappearance of Dsg3 antibodies while on prednisolone and immunosuppressives [11]. Among the 16 cases reported, only 2 had autoantibody profiles in the PF stage that were inconsistent with the clinical and histological diagnosis [3, 5, 7]. …”

“…Among the cases reported previously, 3 were not under any treatment at the onset of the PF stage [2, 10], 2 were on immunosuppressives [1, 11], 3 were on low-dose systemic corticosteroids [3, 7, 8] and 1 was on moderate doses of systemic corticosteroids [9]. There was no mention of treatment in the remaining patients.…”