Rituximab in the management of juvenile pemphigus foliaceus
Abstract
Background
Pemphigus foliaceus (PF) is a blistering disorder most commonly presenting in middle age. As PF is restricted to the superficial epidermis, it is considered more benign than other pemphigus diseases. However, progression to severe disease is not uncommon. Although rituximab’s efficacy has been well-documented in adults with refractory PF, little data is available on its role in adolescents.
Purpose
We describe a patient with juvenile PF treated with rituximab and review the literature for similar cases.
Methods
PubMed was searched for the terms: antibody, B cells, blistering, CD20, foliaceus, juvenile, pemphigus, rituximab, immunosuppression. As the first reported case of rituximab treated pemphigus was in 2001, only cases from 2001 and after were included. Juvenile PF was defined as disease diagnosis between ages 12-17.
Results
Five cases have been reported. The indication for rituximab in most cases was refractory PF unresponsive to systemic glucocorticoids and non-steroidal adjuvant therapies. All cases demonstrated significant improvement or complete remission and most experienced no adverse events.
Conclusions
Rituximab appears to be both well tolerated and efficacious for refractory juvenile PF. Therefore, it may be considered for severe cases of PF to avoid side effects associated with conventional glucocorticoid therapy.