Refractory Childhood Pemphigoid Successfully Treated with Rituximab

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.

Infantile Bullous Pemphigoid Treated Using Intravenous Immunoglobulin: Case Report and Review of the Literature

“…Although IBP usually responds well to first‐line therapy with systemic corticosteroids, resistant cases have been reported. A literature search revealed anecdotal cases of IBP treated using IVIG (Table ) . Variable dosages (200 mg/kg/day to 1 g/kg/day), infusion courses (2‐ to 5‐day courses), and intervals (2–5 wks) were used, probably reflecting the heterogeneity of institutional or individual preferences regarding IVIG treatment in general.…”

Childhood bullous pemphigoid: a report of three cases in China

“…Childhood BP is very rare. There have been fewer than 100 cases of childhood BP reported . The pathogenesis of BP has not yet been fully clarified, but it is widely accepted that BP is strongly correlated with some medications.…”

“…Other treatments that have been used include potent topical steroids alone, particularly in childhood localized vulvar disease . In refractory cases, IVIg and rituximab have been used successfully …”

Bullöses Pemphigoid

“…Dabei sollten vor allem Aza thioprin [11,12], Mycophenolat-Mofetil [12], Methotrexat [13,14], Dapson [15,16] und Doxycyclin [17] genannt werden. Vereinzelte erfolgreiche Behandlungsversuche mit Ciclosporin A [18], Ciclophosphamid [19] und Rituximab [20][21][22] wurden auch berichtet, wobei diese Substanzen nicht im Regelfall zur Behandlung des bullösen Pemphigoids empfohlen werden [9].…”