Published in:
Open Access
01-12-2011 | Case Report
Brain cavernomas associated with en coup de sabre linear scleroderma: Two case reports
Authors:
Emily T Fain, Melissa Mannion, Elena Pope, Daniel W Young, Ronald M Laxer, Randy Q Cron
Published in:
Pediatric Rheumatology
|
Issue 1/2011
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Abstract
Linear scleroderma is a form of localized scleroderma that primarily affects the pediatric population. When it occurs on the scalp or forehead, it is termed "en coup de sabre". In the en coup de sabre subtype, many extracutaneous associations, mostly neurological, have been described. A patient with linear scleroderma en coup de sabre was noted to have ipsilateral brain cavernomas by magnetic resonance imaging. Using a worldwide pediatric rheumatology electronic list-serve, another patient with the same 2 conditions was identified. These two patients are reported in this study. Consideration of neuroimaging studies to disclose abnormal findings in patients with linear scleroderma en coup de sabre is important for potentially preventing and treating neurological manifestations associated with this condition.