Bilateral linear scleroderma en coup de sabre associated with facial atrophy and neurological complicationsReport as inadecuate




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BMC Dermatology

, 1:9

First Online: 04 December 2001Received: 12 November 2001Accepted: 04 December 2001

Abstract

BackgroundLinear scleroderma -en coup de sabre- LSCS usually affects one side of the face and head in the frontoparietal area with band-like indurated skin lesions. The disease may be associated with facial hemiatrophy. Various ophthalmological and neurological abnormalities have been observed in patients with LSCS. We describe an unusual case of LSC.

Case presentationA 23 year old woman presented bilateral LSCS and facial atrophy. The patient had epileptic seizures as well as oculomotor and facial nerve palsy on the left side which also had pronounced skin involvement. Clinical features of different stages of the disease are presented.

ConclusionsThe findings of the presented patient with bilateral LSCS and facial atrophy provide further evidence for a neurological etiology of the disease and may also indicate that classic progressive facial hemiatrophy Parry-Romberg syndrome and LSCS actually represent different spectra of the same disease.

List of abbreviationsLSCSlinear scleroderma -en coup de sabre-

PRSParry-Romberg syndrome

Electronic supplementary materialThe online version of this article doi:10.1186-1471-5945-1-9 contains supplementary material, which is available to authorized users.

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Author: Thilo Gambichler - Alexander Kreuter - Klaus Hoffmann - Falk G Bechara - Peter Altmeyer - Thomas Jansen

Source: https://link.springer.com/







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