Serum Levels of Autoantibodies to Desmoglein 3 in Patients with Therapy-resistant Pemphigus Vulgaris Successfully Treated with Adjuvant Intravenous Immunoglobulins

The mainstay of treatment of pemphigus vulgaris is systemic corticosteroids. Intravenous immunoglobulins have been reported as an adjuvant corticosteroid-sparing regimen in recalcitrant pemphigus vulgaris. The purpose of the study was to monitor disease activity, serum levels of autoantibodies and doses of oral corticosteroids in 4 patients with recalcitrant pemphigus vulgaris adjuvantly treated with intravenous immunoglobulins (2 g kg ^-1 monthly). After initiation of intravenous immunoglobulins, all patients showed clinical improvement and a decrease in autoantibody serum levels, as detected by both indirect immunofluorescence microscopy and ELISA. Corticosteroids and immunosuppressants could be reduced and even discontinued in one patient. In 3 patients, intravenous immunoglobulins were discontinued after 12 cycles. Subsequently, new blisters developed and autoantibody levels rose again. After re-initiation of intravenous immunoglobulins, in 2 patients, the condition quickly improved again, along with a decrease in autoantibody serum levels. It is concluded that the administration of intravenous immunoglobulins was associated with a decrease in circulating autoantibodies and clinical improvement in our patients.