Prospective Study of Pulse Therapy in Childhood Pemphigus Disorders.

Abstract

Background:Pemphigus disorders are a group of immunobullous diseases affecting skin and/or mucus membranes. Dexamethasone cyclophosphamide pulse (DCP)/dexamethasone only pulse (DOP) therapy has shown promising results in the management of pemphigus group of diseases in adults.Aim:To evaluate the outcome of pulse therapy (PT) in pediatric cases diagnosed with pemphigus vulgaris (PV).Materials and Methods:Prospective study of 12 pediatric cases of PV from 2010 to 2015 and treated with PT in Gandhi Hospital. The patients were treated with DOP therapy, with a dose of 50 mg of dexamethasone in 250 ml of 5% dextrose in pediatric patients below the age of 12 years and 100 mg of dexamethasone in 500 ml of 5% dextrose for above 12 years, for three consecutive days. No interpulse steroids or rituximab were given to any patients in our study.Results:Out of 12 cases, 10 were female and 2 were male children. Four cases were below the age of 12 years and 8 cases were above the age of 12 years. The lowest age was 11 years female and highest age was 16 years male child. Average duration of illness is between 4 and 6 months. Six cases completed three phases, four cases were in phase II cycle 7, and two cases were in phase IV. In majority of cases clinical improvement was observed between 2 and 4 pulses in phase I. No significant adverse effects were observed in any case except in two cases who developed headache, shivering, and nausea in first one to two pulses of phase I and managed conservatively.Conclusion:Our study shows that PT gives good response in the management of pemphigus in children, in terms of remission and side effects. There was no significant major adverse effect as observed in adults. In low-resource centers such as government institution, PT would be one option. Further studies and long-term follow-up are required to weigh the risks and benefits of PT in pediatric age group.