Citation, DOI, disclosures and article data
Citation:
Gaillard F, Thibodeau R, Bell D, et al. Linear scleroderma. Reference article, Radiopaedia.org (Accessed on 07 Mar 2025) https://doi.org/10.53347/rID-1585
Linear scleroderma, also known as scleroderma en coup de sabre, 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 parenchyma beneath the skin lesion, focal atrophy and blurring of the gray-white matter interface can be identified Calcification and cerebral microhemorrhages have also been reported 1,5.
History and etymology
"En coup de sabre" alludes to the appearance of a frontoparietal scar as if one had been struck by a sabre (French for a heavy cavalry sword with a curved blade). Coup is French for a blow (as in a blow to the head, and seen in contrecoup injury) 4.
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1. Appenzeller S, Montenegro M, Dertkigil S et al. Neuroimaging Findings in Scleroderma En Coup De Sabre. Neurology. 2004;62(9):1585-9. doi:10.1212/01.wnl.0000124518.25087.18 - Pubmed
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2. Katz K. Frontal Linear Scleroderma (En Coup De Sabre). Dermatol Online J. 2003;9(4):10. - Pubmed
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3. Stone J, Franks A, Guthrie J, Johnson M. Scleroderma "En Coup De Sabre": Pathological Evidence of Intracerebral Inflammation. J Neurol Neurosurg Psychiatry. 2001;70(3):382-5. doi:10.1136/jnnp.70.3.382 - Pubmed
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4. Editors of Chambers. The Chambers Dictionary. (2006) ISBN: 9780550101853 - Google Books
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5. Legendre L, Cuinat L, Curot J, Tanchoux F, Bonneville F, Mazereeuw-Hautier J. [Facial Linear Scleroderma Associated with Neurological Abnormalities Relating to Microangiopathy]. Ann Dermatol Venereol. 2016;143(12):831-5. doi:10.1016/j.annder.2016.02.032 - Pubmed
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