Positive clinical outcome in a patient with recalcitrant bullous pemphigoid treated with rituximab and intravenous immunoglobulin.

A 41-year-old white man was treated for bullous pemphigoid (BP) for 4 years, using high-dose prednisone as well as ciclosporin and mycophenolate mofetil. Sustained clinical improvement was not observed. He suffered several serious side effects. Consequently, he was treated with a combination of rituximab (RTX) and intravenous immunoglobulin (IVIg). He received 12 infusions of RTX in 6 months and monthly IVIg until the end of the therapy. Within 5 weeks of this therapy, appearance of new lesions ceased. Within 8 weeks, all previous lesions resolved and previous medications were discontinued. No hospitalizations, relapses, infections or other serious adverse events occurred. The high levels of pathogenic autoantibody decreased and have remained undetectable. After three infusions of RTX, CD19+ B cells were undetectable and returned to normal levels within 18 months. The patient remains in complete clinical remission off all systemic therapy and free of disease for a 20-month follow-up.