Treatment of juvenile pemphigus vulgaris with intravenous immunoglobulin therapy.

We report the clinical response and follow-up on eight patients with juvenile pemphigus vulgaris treated with intravenous immunoglobulin. Six Caucasian females and two Caucasian males ages 15 to 18 (mean 15.5) were treated with intravenous immunoglobulin based on a published protocol. The indications were lack of response and development of serious side-effects to conventional therapy in four, lack of response to dapsone in two, and parental choice in two patients. In seven patients, a prolonged clinical remission was achieved. They received a mean of 28.5 cycles of intravenous immunoglobulin in a mean of 43.4 months and were followed for a mean of 29.8 months after discontinuing treatment. The remaining patient responded, but was lost to follow-up. Mean follow-up was 71.7 months. Six patients experienced mild headache, but no serious side-effects were observed in any patient. Intravenous immunoglobulin is a safe biological agent to use in the treatment of juvenile pemphigus vulgaris. It can be used as monotherapy and has the potential to induce and sustain long-term clinical remissions. In these eight patients, it appears that intravenous immunoglobulin is a safe biological agent without serious, immediate, or long-term side effects. Intravenous immunoglobulin is a valuable agent in the treatment of certain cases of juvenile pemphigus vulgaris.