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Treatment of Childhood-Onset Lupus Erythematosus Panniculitis With Rituximab.

Authors
  • Correll, Colleen K1
  • Miller, Daniel D2
  • Maguiness, Sheilagh M3
  • 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.
Type
Published Article
Journal
JAMA dermatology
Publication Date
Feb 12, 2020
Identifiers
DOI: 10.1001/jamadermatol.2019.4984
PMID: 32049306
Source
Medline
Language
English
License
Unknown

Abstract

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. To describe the clinical features of childhood-onset LEP and report on the use of rituximab in treatment-refractory childhood-onset LEP. 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. Rituximab therapy for childhood-onset LEP. 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. 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. 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.

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