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MRI abnormalities preceded a rheumatological diagnosis in 7 (28%). Susceptibility lesions were noted in 11 (44%), and 8 (73%) of these patients endorsed a history of headaches. Most lesions were in the supratentorial compartment, did not enhance, and were stable at 1-year follow up imaging. Of those with progression, susceptibility findings were present at baseline. Conclusions Neuroimaging findings in pediatric PRS and ECDS are often supratentorial, stable, unilateral, and ipsilateral to skin findings, and they can precede cutaneous fi