Severe Bullous Pemphigoid in an Infant— Successful Treatment with Rituximab

Abstract

Bullous pemphigoid (BP) is a disease typical of the elderly, and rare in children. With appropriate therapy, the clinical course is usually self-limited. We report on a 5-month-old boy with a severe BP, unresponsive to systemic corticosteroids, intravenous immunoglobulins, dapsone, and cyclosporine A. There is growing evidence for rituximab as a treatment option in refractory autoimmune bullous diseases also in children. We saw a marked improvement in skin lesions within a couple of days after the first dose of rituximab, and blisters disappeared. Monitoring of CD19-positive cells showed a fast decrease to almost zero and a slow recovery within 4 weeks. At this time, new blisters appeared and another dose of rituximab was given. After the second dose of rituximab a long-lasting effect without development of new bullae was observed.

Rituximab for treatment-refractory pemphigus and pemphigoid: A case series of 17 patients

“…So far, rituximab treatment has only been reported in 4 patients with MMP and 10 patients with BP. 4,10- 16 We saw one complete remission off therapy, two complete remissions on therapy, and two partial remissions in MMP and two complete remissions on therapy in BP. Our observation is comparable with previous data that showed healing of all lesions (complete remission on/off therapy) in two of 4 patients with MMP and 7 of 10 with BP.…”

Bullous and mucous membrane pemphigoid show a mixed response to rituximab: experience in seven patients

“…4 There are also case reports for other autoimmune bullous skin disorders, where rituximab therapy successfully treated four patients with MMP and nine with refractory BP (Table 2).…”

Revisiting the role of B cells in skin immune surveillance

“…However, because not all patients respond to these treatments, B cell depletion therapy using Rituximab (with a view to targeting autoreactive B cells) has been evaluated in a limited number of patients. Treatment of immunobullous disorders including pemphigus and bullous pemphigoid with Rituximab resulted in improvement of skin lesions, and has been reported to be associated with a reduction in titers of circulating autoantibodies in some patients, and this was attributed to the systemic depletion of mature CD20 + B cells [75][76][77][78][79][80][81].…”