Linear scleroderma

Last revised by Dalia Ibrahim on 20 Oct 2022

Citation, DOI, disclosures and article data

Citation:

Gaillard F, Ibrahim D, Sharma R, et al. Linear scleroderma. Reference article, Radiopaedia.org (Accessed on 24 Apr 2024) https://doi.org/10.53347/rID-1585

DOI:

https://doi.org/10.53347/rID-1585

Permalink:

https://radiopaedia.org/articles/1585

rID:

1585

Article created:

2 May 2008, Frank Gaillard ◉ ◈

Disclosures:

At the time the article was created Frank Gaillard had no recorded disclosures.

View Frank Gaillard's current disclosures

Last revised:

20 Oct 2022, Dalia Ibrahim ◉

Disclosures:

At the time the article was last revised Dalia Ibrahim had no financial relationships to ineligible companies to disclose.

View Dalia Ibrahim's current disclosures

Revisions:

7 times, by 6 contributors - see full revision history and disclosures

Systems:

Central Nervous System, Head & Neck

Tags:

cases3, reference needed

Synonyms:

Scleroderma en coup de sabre

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.