Long-term follow-up of linear scleroderma en coup de sabre in children with central nervous system involvement

Linear scleroderma en coup de sabre with CNS involvement predominantly affects females and typically involves the left cerebral hemisphere. Characteristic brain MRI findings include ipsilateral supratentorial white matter abnormalities and cyst-like lesions. Combination therapy with systemic corticosteroids and methotrexate is recommended as first-line treatment, while tocilizumab may be beneficial for refractory cases.