Severe bullous pemphigoid in an infant--successful treatment with rituximab.

摘要

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.