Successful treatment of refractory childhood vesiculobullous disorders with rituximab: A study of five cases

Abstract

Introduction: Rituximab, a chimeric monoclonal anti-CD20 antibody, has shown efficacy as an adjuvant in the treatment with refractory vesiculobullous disorders. We, hereby, present a study of five pediatric patients of extensive vesiculobullous disorders showing resistance to conventional therapy of 40 mg of prednisolone daily and treated effectively with rituximab as an adjuvant. Aim of the Study: To study the efficacy, safety, and clinical outcome of rituximab in refractory autoimmune vesiculobullous disorders. Method: Five patients (3: Pemphigus vulgaris, 1: Pemphigus foliaceous, 1: Chronic bullous disease of childhood [CBDC]) were selected for treatment with rituximab after confirmation with tzanck, biopsy, direct immunofluorescence (DIF) and desmoglein (DSG) level. Three hundred milligram intravenous infusion in children over 4–5 h duration. Two doses were given at 15 days interval. DSG 1 and 3 and differential item functioning were repeated after 1-month of the second dose of rituximab. Follow-up (weekly for 1-month, fortnightly for next 2 months). Two patients (1: Pemphigus vulgaris, 1: CBDC) showed relapse after 6 months. So, they were given two more doses of rituximab at an interval of 15 days. Observation: All five patients showed complete remission during the 6 months follow-up period, along with a consensual decline of the serum anti-DSG titers. Conclusion: Rituximab can be considered as an effective adjuvant therapy when treating resistant cases of autoimmune blistering diseases in pediatric patients. However, more number of patients and long-term follow-up is required to draw a definite conclusion.