INTRACEREBRAL INVOLVEMENT IN SCLERODERMA EN COUP DE SABRE - REPORT OFA CASE WITH NEUROPATHOLOGIC FINDINGS

Linear scleroderma en coup de sabre (LScs) is a rare disorder not infr equently associated with neurologic symptoms, notably epilepsy. Howeve r, histopathologic documentation of intracerebral lesions in LScs is v ery limited and the etiology of the central nervous system symptoms ha s therefore never been convincingly established. We describe a 27-year -old woman with LScs and a longstanding history of epilepsy. Radiograp hic studies demonstrated a focal, intraparenchymal lesion in the left frontal lobe directly subjacent to the area of scleroderma on the fore head and scalp. The resected cerebral lesion revealed localized band-l ike sclerosis of the leptomeninges and associated vessels, as well as intraparenchymal calcifications and anomalous, ectatic vessels. These findings suggest that LScs may represent a neurocutaneous syndrome of vascular dysplasia similar to the Sturge-Weber syndrome, rather than a localized form of collagen vascular disease, as suggested by some.