Linear scleroderma "en coup de saber" (LSCS) deformity is a subgroup of scleroderma characterized by unilateral atrophy and depression above the level of the eyebrow. In rare cases where it involves the whole unilateral face it can be mistaken with Parry-Romberg syndrome. Involvement of the trunk and extremities together with facial lesions has been extremely rare in the literature. We report an unusual case of linear scleroderma "en coup de sabre" where there is concomitant involvement of the ipsilateral side of the trunk and lower extremity. The patient's facial deformity is reconstructed with a polyethylene implant.
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