Treatment of Juvenile Pemphigus Vulgaris with Intravenous Immunoglobulins

Summary

Juvenile pemphigus vulgaris (JPV) is a rare autoimmune disease. It is treated by different immunosuppressive drugs, which may have serious side effects. Such problems are most apparent in the therapy of children and adolescents. The use of intravenous immunoglobulins (IVIG) is a safe and effective method and therefore it can replace or at least postpone the initiation of the combined immunosuppressive therapy. The article reports experience with the IVIG therapy of severe JPV in 16-year-old female patient. The double combination of IVIG and prednisone was used. IVIG were administered in a dose of 400 mg/kg/day during three consecutive days with the intervals of 1 – 2 months. Totally 5 cycles were applied. The therapeutic effect was excellent, it started within few days, but it was only temporary and lasted for 1 – 2 months. IVIG therapy enabled the dose of prednisone to be reduced from 50 mg/day to 15 mg/day. Following the cessation of IVIG, the double combination therapy of prednisone and azathioprine was initiated, but it was unsuccessful in controlling the disease. Remission of JPV achieved with triple combination of prednisone (25 mg/day), azathioprine (100 mg/day) and dapsone (50 mg/day) was comparable to that of IVIG and prednisone combination. On the basis of their positive experience the authors recommend to use IVIG also for cases of childhood and juvenile pemphigus, in which this therapy can delay the need for administration of immunosuppressive drugs with potential of serious adverse effects. 

Key words:
juvenile pemphigus vulgaris, childhood pemphigus, intravenous immunoglobulins, therapy, IVIG.