The natural history of pediatric-onset discoid lupus erythematosus - 14/03/15
, Leah Ansell, MD a, Alfred Rademaker, PhD b, Megan L. Curran, MD d, Michael L. Miller, MD d, Annette Wagner, MD c, Brandi M. Kenner-Bell, MD c, Sarah L. Chamlin, MD c, Anthony J. Mancini, MD c, Marisa Klein-Gitelman, MD, MPH d, Amy S. Paller, MS, MD a, cAbstract |
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.
Le texte complet de cet article est disponible en PDF.Key words : autoimmune disease, discoid lupus, pediatric dermatology, systemic lupus erythematosus
Abbreviations used : ACR, CLE, DLE, SLE
Plan
| Dr Arkin is now at Rush University Medical Center, Chicago. Dr Ansell is now at New York Presbyterian Hospital of Columbia University. |
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| Drs Klein-Gitelman and Paller contributed equally to this article as senior authors. |
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| Supported by Society for Pediatric Dermatology Pilot Project Grant 2012. |
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| Conflicts of interest: None declared. |
Vol 72 - N° 4
P. 628-633 - avril 2015 Retour au numéro
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