Linear scleroderma

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.

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

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rID: 1585
Synonyms or Alternate Spellings:

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