title: The natural history of pediatric-onset discoid lupus erythematosus.
abstract: BACKGROUND
  Pediatric discoid lupus erythematosus (DLE) is rare. The risk of progression to systemic lupus erythematosus (SLE) is uncertain.
  
  
  OBJECTIVE
  We sought to determine the risk of progression of pediatric DLE to SLE and to characterize its phenotype.
  
  
  METHODS
  This was a retrospective review of 40 patients with DLE.
  
  
  RESULTS
  Six (15%) of 40 patients presented with DLE as a manifestation of concurrent SLE. Of the remaining 34, 9 (26%) eventually met SLE criteria and 15 (44%) developed laboratory abnormalities without meeting SLE criteria. Only 10 (29%) maintained skin-limited disease. The average age at progression to SLE was 11 years, with greatest risk in the first year after DLE diagnosis. Most (89%) patients with SLE met diagnostic criteria with mucocutaneous disease (discoid lesions, malar rash, oral and nasal ulcers, photosensitivity), positive antibodies, and/or cytopenia without developing end-organ damage over 5 years of median follow-up.
  
  
  CONCLUSIONS
  The study was retrospective.
  
  
  CONCLUSIONS
  In pediatric patients, DLE carries a significant risk of progression to SLE but may predict a milder phenotype of systemic disease. All patients require careful monitoring for SLE, particularly within the first year of diagnosis.
authors: Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address: Lisa.Arkin@gmail.com.
authors: Lisa M
authors: LM
authors: Arkin
authors: Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors: Leah
authors: L
authors: Ansell
authors: Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors: Alfred
authors: A
authors: Rademaker
authors: Division of Rheumatology, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors: Megan L
authors: ML
authors: Curran
authors: Division of Rheumatology, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors: Michael L
authors: ML
authors: Miller
authors: Division of Dermatology, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors: Annette
authors: A
authors: Wagner
authors: Division of Dermatology, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors: Brandi M
authors: BM
authors: Kenner-Bell
authors: Division of Dermatology, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors: Sarah L
authors: SL
authors: Chamlin
authors: Division of Dermatology, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors: Anthony J
authors: AJ
authors: Mancini
authors: Division of Rheumatology, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors: Marisa
authors: M
authors: Klein-Gitelman
authors: Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois; Division of Dermatology, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors: Amy S
authors: AS
authors: Paller
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007668:Kidney / Q000503:physiopathology; D008179:Lupus Erythematosus, Discoid / Q000453:epidemiology / Q000503:physiopathology*; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology; D008181:Lupus Nephritis / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology; D008297:Male; D019226:Oral Ulcer / Q000209:etiology; D015435:Panniculitis, Lupus Erythematosus / Q000175:diagnosis / Q000453:epidemiology; D010641:Phenotype; D012189:Retrospective Studies; D012867:Skin / Q000473:pathology
chemical_list: D001323:Autoantibodies
keywords: autoimmune disease; discoid lupus; pediatric dermatology; systemic lupus erythematosus