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Open Access Highly Accessed Case report

Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications

Thilo Gambichler*, Alexander Kreuter, Klaus Hoffmann, Falk G Bechara, Peter Altmeyer and Thomas Jansen

Author Affiliations

Department of Dermatology, Ruhr-University Bochum, Gudrunstrasse 56, D-44791, Bochum, Germany

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BMC Dermatology 2001, 1:9  doi:10.1186/1471-5945-1-9

Published: 4 December 2001

Abstract

Background

Linear 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 presentation

A 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.

Conclusions

The 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.