Frontal linear scleroderma en coup de sabre associated with epileptic seizure

Linear scleroderma is a rare variant of localised scleroderma, which is usually seen in childhood and during the adolescent period, and can cause severe functional morbidity as well as cosmetic and psychological problems. Although its ethiopathogenesis is yet obscure, autoimmunity, local ischaemia and injuries, vaccination, irradiation, vitamin K injections, Borrelia burgdorferi and Varicella infections have been incriminated. A 4-year-old girl who had been followed up for about 18 months with diagnosis of epilepsy had a colour discolouration and depression that first appeared 1 year ago and then progressed on her left frontal region. Her CT scan showed a thinning in the frontal bone and depression in the frontal region. These findings are described as ‘en coup de sabre’ a rare form of linear scleroderma localised at the frontal region of the scalp. In this paper, we present clinical and radiological findings of a 4-year-old girl with epileptic seizures that started 1 year before the onset of the lesion of linear scleroderma.