Successful treatment of refractory childhood pemphgus vulgaris with anti-CD20 monoclonal antibody (rituximab).

:  Pemphigus vulgarisis an uncommon autoimmune blisteringskin disorder that is particularly rare in children. Immunosuppressive treatment can be challenging. Rituximab(anti- CD20monoclonal antibody) has been used to treat autoimmune disorders by depletion of CD20B cells. Successful rituximabtherapy has been reported in adults with refractory pemphigus vulgaris. We present a girl with childhood pemphigus vulgarisunresponsive to treatment with azathioprine, mycophenolatemofetil, plasmapheresis, and intravenous immunoglobulin with systemic prednisonewho responded to treatment with rituximab. She had a corresponding decline in circulating antibodies against desmoglein 1and 3 and a decline in diphtheriaand tetanus-specific antibody titers.