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 language | English (US) |
|---|---|
| Pages (from-to) | 628-633 |
| Number of pages | 6 |
| Journal | Journal of the American Academy of Dermatology |
| Volume | 72 |
| Issue number | 4 |
| DOIs | |
| State | Published - Jan 1 2015 |
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Keywords
- Key words autoimmune disease
- discoid lupus
- pediatric dermatology
- systemic lupus erythematosus
ASJC Scopus subject areas
- Dermatology
- Medicine(all)
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The natural history of pediatric-onset discoid lupus erythematosus. / Arkin, Lisa M.; Ansell, Leah; Rademaker, Alfred W; Curran, Megan L; Miller, Michael L; Wagner, Annette; Kenner-Bell, Brandi M; Chamlin, Sarah L; Mancini, Anthony J; Klein-Gitelman, Marisa S; Paller, Amy.
In: Journal of the American Academy of Dermatology, Vol. 72, No. 4, 01.01.2015, p. 628-633.Research output: Contribution to journal › Article
TY - JOUR
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
UR - http://www.scopus.com/inward/record.url?scp=84929515277&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84929515277&partnerID=8YFLogxK
U2 - 10.1016/j.jaad.2014.12.028
DO - 10.1016/j.jaad.2014.12.028
M3 - Article
VL - 72
SP - 628
EP - 633
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
SN - 0190-9622
IS - 4
ER -