title: Long-term results of rituximab-intravenous immunoglobulin combination therapy in patients with epidermolysis bullosa acquisita resistant to conventional therapy.
abstract: BACKGROUND
  Epidermolysis bullosa acquisita (EBA) is a rare subepidermal bullous disease. Long-term remission in this disease is difficult using current treatments, unlike that in patients with other autoimmune bullous diseases.
  
  
  OBJECTIVE
  We retrospectively evaluated the effectiveness and side effects of rituximab-intravenous immunoglobulin (IVIg) combination treatment in five patients with EBA resistant to conventional treatment.
  
  
  METHODS
  Rituximab (375 mg/m2) was administered for four consecutive weeks to four patients, and their treatment continued with IVIg at a dose of 2 g/kg/month. One patient received two cycles of rituximab for three consecutive weeks, IVIg in the fourth week, followed by monthly IVIg administrations as in the other patients.
  
  
  RESULTS
  The total number of IVIg therapy cycles ranged from 10 to 26 (mean 19.4). Mean skin involvement, mucosal involvement, and disease severity scores decreased after a mean follow-up of 22.6 months (range, 10-28 months). In an analysis performed during months 24-28, the number of CD19-positive B cells was found to be below the normal reference range in four patients.
  
  
  CONCLUSIONS
  This was a retrospective study with a limited number of patients.
  
  
  CONCLUSIONS
  Rituximab-IVIg combination treatment seems to be effective and safe for treating patients with EBA resistant to conventional treatments.
authors.affiliation: a Department of Dermatology , Ankara Numune Training and Research Hospital , Ankara , Turkey.
authors.forename: Ayse
authors.initials: A
authors.lastname: Oktem
authors.affiliation: b Department of Dermatology, Faculty of Medicine , Ankara University , Ankara , Turkey.
authors.forename: Bengu Nisa
authors.initials: BN
authors.lastname: Akay
authors.affiliation: b Department of Dermatology, Faculty of Medicine , Ankara University , Ankara , Turkey.
authors.forename: Ayse
authors.initials: A
authors.lastname: Boyvat
authors.affiliation: b Department of Dermatology, Faculty of Medicine , Ankara University , Ankara , Turkey.
authors.forename: Nihal
authors.initials: N
authors.lastname: Kundakci
authors.affiliation: b Department of Dermatology, Faculty of Medicine , Ankara University , Ankara , Turkey.
authors.forename: Cengizhan
authors.initials: C
authors.lastname: Erdem
authors.affiliation: b Department of Dermatology, Faculty of Medicine , Ankara University , Ankara , Turkey.
authors.forename: Seher
authors.initials: S
authors.lastname: Bostancı
authors.affiliation: b Department of Dermatology, Faculty of Medicine , Ankara University , Ankara , Turkey.
authors.forename: Hatice
authors.initials: H
authors.lastname: Sanli
authors.affiliation: b Department of Dermatology, Faculty of Medicine , Ankara University , Ankara , Turkey.
authors.forename: Pelin
authors.initials: P
authors.lastname: Kocyigit
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000188:drug therapy; D016107:Epidermolysis Bullosa Acquisita / Q000188:drug therapy*; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage* / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D000069283:Rituximab / Q000008:administration & dosage*
chemical_list: D016756:Immunoglobulins, Intravenous; D000069283:Rituximab
keywords: Epidermolysis bullosa acquisita; intravenous immunoglobulin; rituximab