Korkmaz Cengiz, Adapinar Baki, Uysal Seval
Division of Rheumatology, Department of Internal Medicine, Osmangazi University Medical Faculty, Eskişehir, Turkey.
South Med J. 2005 Sep;98(9):940-2. doi: 10.1097/01.smj.0000177355.43001.ff.
Progressive facial hemiatrophy, also known as Parry-Romberg syndrome (PRS), is characterized by slowly progressive atrophy of one side of the face, primarily involving the subcutaneous tissues and fat. Involvement of the central nervous system with impairment of neurologic function occurs infrequently. At present, there is no agreement as to whether PRS is a distinct entity or a clinical variant of linear scleroderma en coup de sabre. The exact reason for PRS has not yet been determined; therefore, no suitable treatment exists. We observed beneficial effects of immunosuppressive agents on neurologic lesions in particular in a patient with PRS who presented with immunoinflammatory findings and neurologic involvement, apart from cutaneous manifestations.
进行性面部半侧萎缩，也称为帕里 - 龙贝格综合征（PRS），其特征是面部一侧缓慢进行性萎缩，主要累及皮下组织和脂肪。中枢神经系统受累及神经功能损害的情况很少见。目前，关于PRS是一种独立的疾病还是线性硬皮病剑伤型的临床变体尚无定论。PRS的确切病因尚未确定；因此，尚无合适的治疗方法。我们观察到免疫抑制剂对神经病变有有益作用，特别是在一名除皮肤表现外还伴有免疫炎症表现和神经受累的PRS患者中。
