title: Treatment of recalcitrant bullous pemphigoid (BP) with a novel protocol: A retrospective study with a 6-year follow-up.
abstract: BACKGROUND
  Bullous pemphigoid is an autoimmune blistering skin disease that predominantly affects the elderly. Conventional therapy using high-dose systemic corticosteroids and immunosuppressive agents can be ineffective in some patients and produce adverse events and relapses. Hence, alternate therapies are required.
  
  
  OBJECTIVE
  The clinical outcomes of patients with extensive, recalcitrant bullous pemphigoid treated with a combination therapy of rituximab (RTX) and intravenous immunoglobulin were evaluated.
  
  
  METHODS
  In this retrospective study, 12 patients (mean age of 68.25 years) unresponsive to previous conventional immunosuppressive therapy, intravenous immunoglobulin, and/or RTX were treated with this combination therapy.
  
  
  RESULTS
  Complete clinical resolution occurred in a mean of 4.6 months and previous systemic therapy was discontinued in 6.2 months. Two patients had a recurrence posttherapy and responded to additional RTX infusions. The remaining 10 patients had no recurrences. Patients were followed up for a mean of 73.8 months after discontinuation of all systemic therapy. All have remained in remission without adverse events for 6 years.
  
  
  CONCLUSIONS
  This was a retrospective study with a small sample size.
  
  
  CONCLUSIONS
  The combination of RTX and intravenous immunoglobulin produced a sustained clinical remission without adverse events, infections, and hospitalizations. This specific combination protocol offers a promising therapy for patients with recalcitrant bullous pemphigoid.
authors: Center for Blistering Diseases, Department of Dermatology, Tufts University School of Medicine, Boston, Massachusetts. Electronic address: arahmedmd@msn.com.
authors: A Razzaque
authors: AR
authors: Ahmed
authors: Center for Blistering Diseases, Department of Dermatology, Tufts University School of Medicine, Boston, Massachusetts.
authors: Shawn
authors: S
authors: Shetty
authors: Institut National de la Santé et de la Recherche Médicale Unité 1138, Université Pierre et Marie Curie, Paris, France.
authors: Srini
authors: S
authors: Kaveri
authors: Division of Hematology and Oncology, Department of Medicine, Tufts Medical Center, Tufts University Medical School, Boston, Massachusetts.
authors: Zachary S
authors: ZS
authors: Spigelman
mesh_terms: D001688:Biological Products / Q000008:administration & dosage* / Q000009:adverse effects; D015331:Cohort Studies; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage* / Q000009:adverse effects; D008297:Male; D010391:Pemphigoid, Bullous / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality; D012008:Recurrence; D012189:Retrospective Studies; D018570:Risk Assessment; D000069283:Rituximab / Q000008:administration & dosage* / Q000009:adverse effects; D012720:Severity of Illness Index; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D001688:Biological Products; D016756:Immunoglobulins, Intravenous; D000069283:Rituximab
keywords: bullous pemphigoid; clinical response; combination therapy; conventional therapy; death; infection; intravenous immunoglobulin; recalcitrant; remission; rituximab