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Published on: October 12, 2012

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

Johannes Schulze1, Peter Bader, Ulrike Henke

  • 1Departments of Pediatrics, J.W. Goethe University, Frankfurt, Germany. johannes.schulze@kgu.de

Pediatric Dermatology
|September 16, 2008
PubMed

Insights

Bullous pemphigoid (BP) is rare in children but can be severe. Rituximab effectively treated a severe case unresponsive to other therapies, offering a new option for pediatric autoimmune blistering diseases.

Area of Science:

  • Pediatric Dermatology
  • Autoimmune Blistering Diseases
  • Immunology

Background:

  • Bullous pemphigoid (BP) is a rare autoimmune blistering disease in children, typically affecting the elderly.
  • Standard treatments like corticosteroids, IVIG, dapsone, and cyclosporine A are not always effective.

Observation:

  • A 5-month-old infant presented with severe bullous pemphigoid refractory to multiple conventional therapies.
  • The patient received rituximab for treatment-resistant bullous pemphigoid.

Findings:

  • Marked improvement in skin lesions and blister resolution occurred within days of the first rituximab dose.
  • CD19-positive cell depletion was observed, with relapse coinciding with cell recovery.
  • A second rituximab dose led to a long-lasting therapeutic effect without new blister formation.

Implications:

  • Rituximab demonstrates significant efficacy in treating severe, refractory pediatric bullous pemphigoid.
  • This suggests rituximab is a viable therapeutic option for children with autoimmune bullous diseases.
  • Monitoring CD19+ cells may help guide rituximab retreatment strategies in pediatric BP.

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