title: Treatment of refractory pemphigus with the anti-CD20 monoclonal antibody (rituximab).
abstract: BACKGROUND
  Pemphigus is a severe blistering disorder caused by autoantibodies to desmogleins 1 and 3. Because some patients with pemphigus never enter into remission, new immunosuppressants are warranted. Rituximab is a chimeric monoclonal antibody binding to the CD20 antigen on B cells, which proved to be effective in recalcitrant pemphigus.
  
  
  OBJECTIVE
  To evaluate the efficacy and safety of rituximab in refractory pemphigus and to investigate its effects on the autoantibody profile.
  
  
  METHODS
  Six patients with recalcitrant pemphigus were treated. Rituximab was administered intravenously at a dosage of 375 mg/m2 body surface once weekly for 4 weeks.
  
  
  RESULTS
  Three pemphigus foliaceus patients and 1 with mucocutaneous pemphigus vulgaris (PV) showed complete response over a follow-up period of up to 18 months. In one oral PV, control of the disease was achieved using pulse therapy with cyclophosphamide following rituximab withdrawal. In one PV with vegetating features, good improvement was obtained after 6 rituximab infusions. All patients tolerated the treatment well. Anti-desmoglein autoantibodies significantly decreased only in pemphigus foliaceus.
  
  
  CONCLUSIONS
  This study highlights that rituximab is a valuable drug for refractory pemphigus, although the response of mucous membranes and cutaneous folds may be delayed.
authors: Institute of Dermatological Sciences, University of Milan, IRCCS Ospedale Maggiore Policlinico, Mangiagalli and Regina Elena of Milan, Milan, Italy.
authors: Angelo V
authors: AV
authors: Marzano
authors: Daniele
authors: D
authors: Fanoni
authors: Luigia
authors: L
authors: Venegoni
authors: Emilio
authors: E
authors: Berti
authors: Ruggero
authors: R
authors: Caputo
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D018941:Antigens, CD19 / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D001402:B-Lymphocytes / Q000276:immunology; D051183:Desmoglein 1 / Q000097:blood; D051184:Desmoglein 3 / Q000097:blood; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy*; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018941:Antigens, CD19; D001323:Autoantibodies; C071894:DSG3 protein, human; D051183:Desmoglein 1; D051184:Desmoglein 3; D007155:Immunologic Factors; D000069283:Rituximab