Linear scleroderma (LS) is a very focal form of scleroderma.
It is also known as en coup de sabre, alluding the appearance of a fronto-parietal scar as if one had been struck by a sabre (french), It almost never progresses to systemic scleroderma.
Immediately subjacent to the 'scar', the skull is often thinned. In the brain beneath the skin lesion, focal atrophy and blurring of the gray-white matter interface can be identified, and calcification has been reported.
- 1. Appenzeller, S. "Neuroimaging findings in scleroderma en coup de sabre." Neurology. 2004 May 11;62(9):1585-9
- 2. Kenneth, A. "Frontal linear scleroderma (en coup de sabre)" Dermatology Online Journal 9(4): 10
- 3. Stone, J. "Scleroderma "en coup de sabre": pathological evidence of intracerebral inflammation" J Neurol Neurosurg Psychiatry 2001;70:382-385 ( March )