title: 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.
authors.affiliation: Departments of Pediatrics, J.W. Goethe University, Frankfurt, Germany. johannes.schulze@kgu.de
authors.forename: Johannes
authors.initials: J
authors.lastname: Schulze
authors.forename: Peter
authors.initials: P
authors.lastname: Bader
authors.forename: Ulrike
authors.initials: U
authors.lastname: Henke
authors.forename: Markus A
authors.initials: MA
authors.lastname: Rose
authors.forename: Stefan
authors.initials: S
authors.lastname: Zielen
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D010391:Pemphigoid, Bullous / Q000188:drug therapy* / Q000473:pathology; D000069283:Rituximab
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; D000069283:Rituximab