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Treatment of Childhood-Onset Lupus Erythematosus Panniculitis With Rituximab

Author

Correll CK1, Miller DD2, Maguiness SM3. JAMA Dermatol. 2020 Feb 12. doi: 10.1001/jamadermatol.2019.4984. [Epub ahead of print]

Author Information

1 Division of Rheumatology, Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis.

2 Division of Dermatopathology, Department of Dermatology, University of Minnesota Medical Center, Minneapolis.

3 Division of Pediatric Dermatology, Department of Dermatology, University of Minnesota Medical Center, Minneapolis.

Abstract

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.