Linear scleroderma

Last revised by Dalia Ibrahim on 20 Oct 2022

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.

Linear scleroderma may coexist with progressive facial hemiatrophy (Parry-Romberg syndrome).

Radiographic features

In the brain beneath the skin lesion, focal atrophy and blurring of the gray-white matter interface can be identified, and calcification and cerebral microhemorrhages have been reported 1,5.

History and etymology 

"En coup de saber" alludes to the appearance of a frontoparietal scar as if one had been struck by a saber (French for a heavy cavalry sword with a curved blade). Coup is French for a blow (as in a blow to the head) 4.

ADVERTISEMENT: Supporters see fewer/no ads