Lisa M Arkin1, Leah Ansell2, Alfred Rademaker3, Megan L Curran4, Michael L Miller4, Annette Wagner5, Brandi M Kenner-Bell5, Sarah L Chamlin5, Anthony J Mancini5, Marisa Klein-Gitelman4, Amy S Paller6. 1. Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address: Lisa.Arkin@gmail.com. 2. Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois. 3. Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois. 4. Division of Rheumatology, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois. 5. Division of Dermatology, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois. 6. 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.
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.
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.
Authors: Josef Symon S Concha; Aikaterini Patsatsi; Ann Marshak-Rothstein; Ming-Lin Liu; Animesh A Sinha; Lela A Lee; Joseph F Merola; Ali Jabbari; Johann E Gudjonsson; François Chasset; Paul Jarrett; Benjamin Chong; Lisa Arkin; Anthony P Fernandez; Marzia Caproni; Steven A Greenberg; Hee Joo Kim; David R Pearson; Alisa Femia; Ruth Ann Vleugels; David Fiorentino; Manabu Fujimoto; Joerg Wenzel; Victoria P Werth Journal: J Invest Dermatol Date: 2018-09-19 Impact factor: 8.551