Bergler-Czop Beata, Lis-Swiety Anna, Brzezińska-Wcisło Ligia
Department of Dermatology Silesian Medical University in Katowice, Francuska Street 20/24, 40-027 Katowice, Poland.
BMC Neurol. 2009 Jul 27;9:39. doi: 10.1186/1471-2377-9-39.
Hemifacial atrophy (Parry-Romberg syndrome) is a relatively rare disease. The etiology of the disease is not clear. Some authors postulate its relation with limited scleroderma linearis. Linear scleroderma "en coup de sabre" is characterized by clinical presence of most commonly one-sided linear syndrome. In a number of patients, neurological affection is the medium of the disease. The treatment of both scleroderma varieties is similar to the treatment of limited systemic sclerosis.
We present two cases of a disease: a case of a 49-year-old woman with a typical image of hemifacial atrophy, without any changes of the nervous system and a case of a 33-year-old patient with an "en coup de sabre" scleroderma and with CNS tumor.
We described typical cases of a rare diseases, hemifacial atrophy and "en coup de sabre" scleroderma. In the patient diagnosed with Parry-Romberg syndrome, with Borrelia burgdoferi infection and with minor neurological symptoms, despite a four-year case history, there was a lack of proper diagnosis and treatment.In the second patient only skin changes without any neurological symptoms could be observed and only a precise neurological diagnosis revealed the presence of CNS tumor.
半侧面部萎缩（帕里-罗姆伯格综合征）是一种相对罕见的疾病。该病的病因尚不清楚。一些作者推测其与局限性线状硬皮病有关。线状硬皮病“剑伤样”的特征是临床上最常见为单侧线状综合征。在一些患者中，神经受累是疾病的媒介。这两种硬皮病的治疗与局限性系统性硬化症的治疗相似。
我们展示了该疾病的两个病例：一例是一名49岁女性，具有典型的半侧面部萎缩影像，神经系统无任何变化；另一例是一名33岁患者，患有“剑伤样”硬皮病且伴有中枢神经系统肿瘤。
我们描述了罕见疾病半侧面部萎缩和“剑伤样”硬皮病的典型病例。在被诊断为帕里-罗姆伯格综合征、感染伯氏疏螺旋体且有轻微神经症状的患者中，尽管有四年病史，但仍缺乏正确的诊断和治疗。在第二名患者中，仅观察到皮肤变化而无任何神经症状，只有精确的神经诊断才发现存在中枢神经系统肿瘤。
