Linear scleroderma ‘en coup de sabre’
Abstract
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.
Key Points
Objective
To investigate the clinical presentation and management of adult-onset linear scleroderma ‘en coup de sabre’.
Methods
- Clinical assessment of a 43-year-old woman with a forehead lesion and epilepsy.
- MRI brain scan to evaluate neurological changes.
- Skin biopsy to confirm the diagnosis of scleroderma.
Results
- Patient presented with a scar-like lesion and epilepsy.