Linear scleroderma, also known as scleroderma en coup de saber, is a very focal form of scleroderma classically characterized by a linear band of atrophy involving the frontal or frontoparietal scalp and subjacent thinned calvaria associated with ipsilateral focal brain abnormalities.
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.
Linear scleroderma may coexist with progressive facial hemiatrophy (PFHA) (also named Parry-Romberg syndrome).
History and etymology
"En coup de saber" alludes to the appearance of a fronto-parietal scar as if one had been struck by a saber (French for a heavy cavalry sword with a curved blade).
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