title: Recommendations for the use of rituximab (anti-CD20 antibody) in the treatment of autoimmune bullous skin diseases.
abstract: Autoimmune bullous skin disorders are induced by autoantibodies against distinct adhesion complexes of the epidermal and dermal-epidermal junction. Since most of these disorders are characterized by a severe, potentially lethal course,they require long-term immunosuppressive treatment to reduce the de novo synthesis of pathogenic autoantibodies by B lymphocytes. Rituximab, a chimeric monoclonal antibody against CD20 on B lymphocytes, has shown promise in several case reports or cohort studies in the treatment of paraneo-plastic pemphigus,refractory cases of pemphigus vulgaris and foliaceus and in other autoimmune bullous disorders. Treatment with rituximab leads to depletion of pathogenic B-cells which may last up to 12 months resulting in a reduction of plasma cells secreting pathogenic autoantibodies. Rituximab is usually administered in an adjuvant setting at a dose of 375 mg/m(2) i.v.in weekly intervals for four consecutive weeks in addition to the standard immunosuppressive treatment. The present consensus statement of German-speaking dermatologists, rheumatologists and oncologists summarizes and evaluates the current evidence for the use and mode of application of rituximab in autoimmune bullous skin disorders.
authors.affiliation: Department of Dermatology and Allergology, University Clinic of Giessen and Marburg GmbH, Marburg, Germany. hertl@med.uni-marburg.de
authors.forename: Michael
authors.initials: M
authors.lastname: Hertl
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
authors.forename: Luca
authors.initials: L
authors.lastname: Borradori
authors.forename: Leena
authors.initials: L
authors.lastname: Bruckner-Tuderman
authors.forename: Harald
authors.initials: H
authors.lastname: Burckhard
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Eming
authors.forename: Andreas
authors.initials: A
authors.lastname: Engert
authors.forename: Matthias
authors.initials: M
authors.lastname: Goebeler
authors.forename: Silke
authors.initials: S
authors.lastname: Hofmann
authors.forename: Nicolas
authors.initials: N
authors.lastname: Hunzelmann
authors.forename: Franz
authors.initials: F
authors.lastname: Karlhofer
authors.forename: Ocko
authors.initials: O
authors.lastname: Kautz
authors.forename: Undine
authors.initials: U
authors.lastname: Lippert
authors.forename: Andrea
authors.initials: A
authors.lastname: Niedermeier
authors.forename: Martin
authors.initials: M
authors.lastname: Nitschke
authors.forename: Martin
authors.initials: M
authors.lastname: Pfütze
authors.forename: Marcel
authors.initials: M
authors.lastname: Reiser
authors.forename: Christian
authors.initials: C
authors.lastname: Rose
authors.forename: Enno
authors.initials: E
authors.lastname: Schmidt
authors.forename: Iakov
authors.initials: I
authors.lastname: Shimanovich
authors.forename: Michael
authors.initials: M
authors.lastname: Sticherling
authors.forename: Sonja
authors.initials: S
authors.lastname: Wolff-Franke
mesh_terms: D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D058846:Antibodies, Monoclonal, Murine-Derived; D001327:Autoimmune Diseases / Q000188:drug therapy*; D003880:Dermatology / Q000592:standards*; D004334:Drug Administration Schedule; D006801:Humans; D017410:Practice Guidelines as Topic; D000069283:Rituximab; D012872:Skin Diseases, Vesiculobullous / Q000188:drug therapy*
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab