title: Successful treatment of refractory childhood pemphgus vulgaris with anti-CD20 monoclonal antibody (rituximab).
abstract: Pemphigus vulgaris is an uncommon autoimmune blistering skin disorder that is particularly rare in children. Immunosuppressive treatment can be challenging. Rituximab (anti-CD20 monoclonal antibody) has been used to treat autoimmune disorders by depletion of CD20 B cells. Successful rituximab therapy has been reported in adults with refractory pemphigus vulgaris. We present a girl with childhood pemphigus vulgaris unresponsive to treatment with azathioprine, mycophenolate mofetil, plasmapheresis, and intravenous immunoglobulin with systemic prednisone who responded to treatment with rituximab. She had a corresponding decline in circulating antibodies against desmoglein 1 and 3 and a decline in diphtheria and tetanus-specific antibody titers.
authors.affiliation: Division of Dermatology, Duke University Medical Center, Durham, North Carolina 27710, USA.
authors.forename: Heidi H
authors.initials: HH
authors.lastname: Kong
authors.forename: Neil S
authors.initials: NS
authors.lastname: Prose
authors.forename: Russell E
authors.initials: RE
authors.lastname: Ware
authors.forename: Russell P
authors.initials: RP
authors.lastname: Hall
mesh_terms: D000293:Adolescent; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20 / Q000276:immunology; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010392:Pemphigus / Q000175:diagnosis / Q000188:drug therapy*; D000069283:Rituximab; D017211:Treatment Failure; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab