Arif Tasleem, Fatima Rafiya, Sami Marwa
Ellahi Medicare Clinic, Srinagar, Kashmir, India.
Department of Dermatology, Tadawi General Hospital, Dammaam, Saudi Arabia.
Acta Dermatovenerol Alp Pannonica Adriat. 2020 Dec;29(4):193-199.
Parry-Romberg syndrome (PRS) is a rare disorder of uncertain etiology that is characterized by progressive atrophy of the soft and hard tissues of face, typically occurring in the first 2 decades of life. It is more commonly seen in females. The disease progresses slowly with gradual atrophy, frequently associated with neurological, ophthalmological, and other system involvement, resulting in secondary complications. The severity of deformity varies depending on the age of onset of disease. Those in whom the disease starts at an earlier age will have more severe deformity. Due to the visible facial deformity, such patients usually suffer from social and psychological trauma. Management is mainly cosmetic, which is carried out after disease progression has stopped and stabilized. This brief review describes PRS in detail and compares it with linear morphea en coup de sabre (ECDS), its close differential, which is likely to be a milder variant sharing the same spectrum of disease.
帕里-罗姆伯格综合征（PRS）是一种病因不明的罕见疾病，其特征是面部软组织和硬组织进行性萎缩，通常发生在生命的前20年。女性更为常见。该病随着逐渐萎缩而缓慢进展，常伴有神经、眼科和其他系统受累，导致继发性并发症。畸形的严重程度因疾病发病年龄而异。发病年龄较早的患者畸形会更严重。由于面部畸形明显，这类患者通常会遭受社会和心理创伤。治疗主要是美容治疗，在疾病进展停止并稳定后进行。本简要综述详细描述了PRS，并将其与其密切鉴别诊断——线状硬皮病剑伤型（ECDS）进行比较，后者可能是同一疾病谱中较温和的变体。
