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Linear scleroderma ‘en coup de sabre’ (LSCS) has been reported in association with intracranial abnormalities. We report the case of an 11‐year‐old boy with LSCS who presented with recurrent headaches. Cranial magnetic resonance imaging (MRI) and angiography were consistent with the diagnosis of a cerebral vasculitis. In addition, retinal examination showed an exudative inflammatory lesion consistent with vasculitis.
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