Ozturk Serdar, Acarturk Tahsin Oguz, Yapici Kerim, Sengezer Mustafa
J Craniofac Surg. 2006 Jul;17(4):696-701. doi: 10.1097/00001665-200607000-00016.
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.
线性硬皮病“剑伤状”(LSCS)畸形是硬皮病的一个亚组,其特征为单侧眉毛上方萎缩和凹陷。在极少数累及整个单侧面部的情况下,它可能被误诊为帕里 - 罗姆伯格综合征。在文献中,躯干和四肢与面部病变同时受累极为罕见。我们报告一例罕见的线性硬皮病“剑伤状”病例,其同侧躯干和下肢同时受累。患者的面部畸形通过聚乙烯植入物进行了重建。