Refractory childhood pemphigoid successfully treated with rituximab
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Published on: June 17, 2025
Irene Fuertes1, Jesús Luelmo, Lorena Leal
1Department of Dermatology, Hospital Parc Taulí de Sabadell, Universitat Autonoma de Barcelona, Sabadell, Barcelona, Spain.
Insights
Childhood bullous pemphigoid (CBP) is rare but can be severe. Rituximab effectively treated a severe infant case, allowing steroid reduction, though optimal pediatric dosing remains unestablished.
Area of Science:
- Dermatology
- Autoimmune Disorders
- Pediatrics
Background:
- Bullous pemphigoid is a rare autoimmune blistering disease primarily affecting the elderly.
- Childhood bullous pemphigoid (CBP) is exceptionally rare, with fewer than 100 reported cases.
- CBP typically has a good prognosis, but severe, refractory cases can occur.
Observation:
- This report details a severe case of CBP in an infant.
- The infant's condition was refractory to multiple treatments, including azathioprine and intravenous immunoglobulin combined with high-dose prednisolone.
- Rituximab administration resulted in a complete clinical response and enabled significant steroid tapering.
Findings:
- Rituximab demonstrated high efficacy in treating severe, treatment-refractory childhood bullous pemphigoid.
- The case highlights rituximab's potential as a steroid-sparing agent in pediatric autoimmune blistering diseases.
- Complete clinical remission was achieved with rituximab, facilitating substantial reduction in corticosteroid therapy.
Implications:
- This case suggests rituximab may be a valuable therapeutic option for severe pediatric bullous pemphigoid.
- Further research is needed to establish optimal dosing and frequency of rituximab for pediatric blistering diseases.
- The findings contribute to understanding treatment strategies for rare autoimmune conditions in children.
Abstract:
Bullous pemphigoid is an acquired autoimmune blistering disorder affecting mostly elderly people that is rare in children. There have been fewer than 100 cases of childhood bullous pemphigoid reported. Childhood bullous pemphigoid (CBP) generally has a good prognosis. Remission is usually achieved within several weeks to a few months, and relapses are rare, although refractory cases can occur, and the disease may be life threatening, particularly when lesions generalize. Herein we describe the case of an infant with severe CBP refractory to multiple treatments. In our case, therapy with azathioprine and intravenous immunoglobulin resulted in a slight clinical response, but only combined with high doses of prednisolone. Administration of rituximab led to a complete clinical response and allowed almost complete steroid tapering. There are no current guidelines indicating what doses and frequency of rituximab are safest or most effective in children with blistering diseases.