Treatment of Childhood-Onset Lupus Erythematosus Panniculitis With Rituximab

This case series describes the clinical features of childhood-onset lupus erythematosus panniculitis and reports on the use of rituximab for patients with treatment-refractory, childhood-onset disease. Question What is known about the clinical and histopathologic features and treatment of childhood-onset lupus erythematosus panniculitis? Findings In this case series describing 4 patients with childhood-onset lupus erythematosus panniculitis refractory to many therapies, improvement was noted after treatment with rituximab. Meaning Lupus erythematosus panniculitis is a rare and disfiguring cutaneous disease in childhood with no criterion standard therapies; however, the findings from this case series suggest that rituximab may be an effective and well-tolerated treatment. Importance Childhood-onset lupus erythematosus panniculitis (LEP) is a rare and chronic disfiguring disease. A paucity of literature exists on the clinical manifestations of this disease and how best to treat it. Objectives To describe the clinical features of childhood-onset LEP and report on the use of rituximab in treatment-refractory childhood-onset LEP. Design, Setting, and Participants A retrospective, observational case series study was conducted of 4 patients with childhood-onset LEP who presented to a single-center, tertiary care clinic with pediatric dermatology and pediatric rheumatology clinics between July 1, 2014, and July 1, 2018, and were treated with rituximab. A literature review was conducted of the clinical features and treatment of childhood-onset LEP. Exposure Rituximab therapy for childhood-onset LEP. Main Outcomes and Measures Reduction in the number and size of erythematous and tender subcutaneous nodules (both visually and by palpation), reduction of facial atrophy (documented with serial photography), and tolerability of rituximab at 2 to 22 months after initiation of therapy. Results Four patients (3 male; mean [SD] age at treatment, 15 [5.9] years) with refractory childhood-onset LEP were successfully treated with rituximab. All patients had a rapid and sustained response to therapy with rituximab. One patient (25%) had minor infusion reactions; otherwise, treatment was well tolerated. Conclusions and Relevance This case series suggests that rituximab may hold promise as a treatment for refractory, childhood-onset LEP. Larger, prospective studies are needed to validate these findings; however, given the rarity of disease, large studies may be difficult to conduct.