Jun Jae Hun, Kim Ho Youn, Jung Han Jin, Lee Weon Ju, Lee Seok-Jong, Kim Do Won, Kim Moon Bum, Kim Byung Soo
Department of Dermatology, Kyungpook National University School of Medicine, Daegu, Korea.
Ann Dermatol. 2011 Aug;23(3):342-7. doi: 10.5021/ad.2011.23.3.342. Epub 2011 Aug 6.
Parry-Romberg syndrome (PRS) is a relatively rare degenerative disorder that is poorly understood. PRS is characterized by slowly progressing atrophy affecting one side of the face, and is frequently associated with localized scleroderma, especially linear scleroderma, which is known as en coup de sabre. This is a report of the author's experiences with PRS accompanying en coup de sabre, and a review of the ongoing considerable debate associated with these two entities. Case 1 was a 37-year-old woman who had right hemifacial atrophy with unilateral en coup de sabre for seven years. Fat grafting to her atrophic lip had been conducted, and steroid injection had been performed on the indurated plaque of the forehead. Case 2 was a 29-year-old woman who had suffered from right hemifacial atrophy and bilateral en coup de sabre for 18 years. Surgical corrections such as scapular osteocutaneous flap and mandible/maxilla distraction showed unsatisfying results.
帕里-罗姆伯格综合征（PRS）是一种相对罕见且了解甚少的退行性疾病。PRS的特征是一侧面部缓慢进行性萎缩，常与局限性硬皮病相关，尤其是线状硬皮病，即所谓的“剑伤样硬皮病”。本文报告了作者对伴有“剑伤样硬皮病”的PRS的诊治经验，并对这两种疾病相关的持续激烈争论进行了综述。病例1是一名37岁女性，右侧面部萎缩伴单侧“剑伤样硬皮病”7年。曾对其萎缩的唇部进行脂肪移植，并对前额的硬结斑块进行类固醇注射。病例2是一名29岁女性，患有右侧面部萎缩和双侧“剑伤样硬皮病”18年。肩胛骨皮瓣和下颌骨/上颌骨牵张等手术矫正效果不佳。
