Linear scleroderma 'en coup de sabre'

Linear scleroderma 'en coup de sabre' is a rare condition characterised by inflammation and fibrosis of the skin and underlying tissues; it rarely presents in adults. The adult-onset form seldom has extracutaneous manifestations, but those that occur are usually neurological. A 43-year-old woman with epilepsy had a scar-like lesion on her forehead. The MR brain scan showed focal cortical atrophy and white matter changes on the same side as the skin lesion. Skin biopsy identified hyaline sclerosis of the reticular dermis consistent with scleroderma. Her clinical condition stabilised on mycophenolate mofetil. Although uncommon in adults, it is important to consider this rare disease in adults with scalp lesions who have epileptic seizures.