A case of multiple cerebral cavernomas associated with en coup de sabre scleroderma

Abstract

Linear scleroderma is a form of localized scleroderma that presents as band-like sclerotic lesions. When it occurs on the scalp or forehead, it is called "en coup de sabre scleroderma" (ECDS). Extracutaneous associations, mostly neurologic, have been described with ECDS.We report a patient with ECDS associated with multiple cerebral cavernomas. This serves to highlight the importance of neuroimaging and multidisciplinary team involvement in patients with ECDS. Pediatric Dermatology BRIEF REPORTmalformations of the central nervous system. They consist of vessels that lack smooth muscle support and are prone to rupture, leading to hemorrhage, stroke, seizures, or death. 3 The pathogenesis of neurologic dysfunction is unclear. The most accepted theory is the "neurovasculitis hypothesis" based on focal MRI and angiographic evidence of CNS vascular changes.We report a patient with extensive ECDS associated with multiple cerebral cavernomas, resulting in cerebral bleeding and seizures.Our case highlights the importance of neuroimaging and multidisciplinary team involvement at the time of diagnosis of ECDS, given that neurologic involvement has been reported in 62% of patients with linear morphea on the scalp. 4 Since neurologic abnormalities can manifest at different points, it is important to provide regular follow-up for patients with ECDS to facilitate early management and prevent lifelong complications. Systemic immunosuppressive therapy should be considered for patients with intracranial abnormalities. D-penicillamine, methylprednisolone, mycophenolate mofetil, and methotrexate have been reported as treatment options for ECDS patients with intractable seizures and to limit intracranial damage. 5 On the other hand, the treatment for the intracranial cavernomas remains surgical. K E Y WO R DS cavernomas, linear morphea Noor Alwash MBBS, MRCP Naila Dinani MBBS, MRCP

Intraparenchymal Hemorrhage in En coup de sabre: A Case Report

“…He did not have correlating neurological symptoms of ECDS. At age 4, he presented with generalized seizures and the occipital intraparenchymal lesion was found to have increased in size [7].…”