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Background: The incidence of systemic lupus in children with discoid lupus is unknown., Objective: This study assessed the baseline characteristics of patients with pediatric discoid lupus erythematosus (pDLE)., Methods: Medical records at 17 sites were reviewed for pediatric dermatology and rheumatology patients with discoid lupus erythematosus. The inclusion criteria were clinical and/or histopathologic diagnosis of discoid lupus erythematosus with an age at onset of <18 years. Baseline data were collected at the first documented visit. Outcomes included diagnosis of systemic lupus erythematosus (SLE) at the baseline visit using the 1997 American College of Rheumatology (primary) and the 2012 Systemic Lupus International Collaborating Clinics (secondary) criteria., Results: Of the >1500 charts reviewed, 438 patients met the inclusion criteria. The cohort was predominantly female (72%) and racially/ethnically diverse. A diagnosis of SLE at the baseline visit (pDLE + SLE) was rendered in 162 (37%) patients using the American College of Rheumatology and in 181 (41%) patients using the Systemic Lupus International Collaborating Clinics criteria. Patients with pDLE + SLE were older at the time of rash onset (median, 12.9 vs 8.9 years; P < .001), with shorter time from discoid lupus erythematosus onset to diagnosis, compared with patients with pDLE-only (median, 2 vs 7 months; P < .001). Patients with pDLE + SLE were more likely to be female (P = .004), with generalized discoid lupus erythematosus and clinically aggressive disease, including end-organ involvement, positive serologies, and higher- titer levels of antinuclear antibodies (P < .001)., Limitations: Retrospective study., Conclusion: A diagnosis of discoid lupus erythematosus in adolescence should prompt thorough screening for SLE., Competing Interests: Conflicts of interest Dr Ardoin is a consultant for Aurinia. Dr Chong is an investigator for Daavlin Corporation, Pfizer Incorporated, Biogen Incorporated, and Amgen Incorporated; is a consultant for Viela Bio, Beacon Bioscience, Bristol Meyers Squibb, EMD Serono, and Principia Biopharma; reports research grants (paid to his institution) from Daavlin Corporation and Biogen Incorporated; reports honoraria from Viela Bio, Beacon Bioscience, Bristol Meyers Squibb, EMD Serono, and Principia Biopharma as a consultant. Dr von Scheven is the President of the Childhood Arthritis and Rheumatology Alliance. Drs Ezeh, Ardalan, Buhr, Nguyen, Ahmed, Barton, Bell, Brandling-Bennett, Castelo-Soccio, Chiu, Co, Lara-Corrales, Cinosun, Curran, Diaz, Elman, Fernandez-Faith, Garcia-Romero, Grossman-Kranseler, Hogeling, Hudson, Hunt, Ibler, Marques, Monir, Oza, Paller, Putterman, Rodriguez-Salgado, Schoch, Truong, Wang, Wine Lee, Vleugels, Klein-Gitelman, Werth, and Arkin have no conflicts of interest to declare., (Copyright © 2022. Published by Elsevier Inc.)