The natural history of pediatric-onset discoid lupus erythematosus

Lisa M. Arkin*, Leah Ansell, Alfred W Rademaker, Megan L Curran, Michael L Miller, Annette Wagner, Brandi M Kenner-Bell, Sarah L Chamlin, Anthony J Mancini, Marisa S Klein-Gitelman, Amy Paller

*Corresponding author for this work

Research output: Contribution to journalArticle

16 Citations (Scopus)

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. Limitations 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.

Original languageEnglish (US)
Pages (from-to)628-633
Number of pages6
JournalJournal of the American Academy of Dermatology
Volume72
Issue number4
DOIs
StatePublished - Jan 1 2015

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Discoid Lupus Erythematosus
Natural History
Systemic Lupus Erythematosus
Pediatrics
Oral Ulcer
Phenotype
Exanthema
Nose
Skin Diseases
Retrospective Studies

Keywords

  • Key words autoimmune disease
  • discoid lupus
  • pediatric dermatology
  • systemic lupus erythematosus

ASJC Scopus subject areas

  • Dermatology
  • Medicine(all)

Cite this

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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. Limitations 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.",
keywords = "Key words autoimmune disease, discoid lupus, pediatric dermatology, systemic lupus erythematosus",
author = "Arkin, {Lisa M.} and Leah Ansell and Rademaker, {Alfred W} and Curran, {Megan L} and Miller, {Michael L} and Annette Wagner and Kenner-Bell, {Brandi M} and Chamlin, {Sarah L} and Mancini, {Anthony J} and Klein-Gitelman, {Marisa S} and Amy Paller",
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T1 - The natural history of pediatric-onset discoid lupus erythematosus

AU - Arkin, Lisa M.

AU - Ansell, Leah

AU - Rademaker, Alfred W

AU - Curran, Megan L

AU - Miller, Michael L

AU - Wagner, Annette

AU - Kenner-Bell, Brandi M

AU - Chamlin, Sarah L

AU - Mancini, Anthony J

AU - Klein-Gitelman, Marisa S

AU - Paller, Amy

PY - 2015/1/1

Y1 - 2015/1/1

N2 - 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. Limitations 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.

AB - 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. Limitations 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.

KW - Key words autoimmune disease

KW - discoid lupus

KW - pediatric dermatology

KW - systemic lupus erythematosus

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