Department of Pediatrics, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan.
Pediatr Neonatol. 2009 Dec;50(6):294-8. doi: 10.1016/S1875-9572(09)60081-4.
Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. The "en coup de sabre" subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. Here, we report one patient with typical linear scleroderma "en coup de sabre" (LSCS). Initially, he presented with refractory partial seizures before the characteristic skins lesion on his head developed. This was a rare case with obvious brain parenchyma involvement. We did not prescribe medication but performed serial brain magnetic resonance imaging follow-up for the intraparenchymal lesion. The atrophic changes of the skin, face and brain remained the same, and his seizures had not worsened at the most recent follow-up. Parry-Romberg syndrome, a very similar condition, should be differentiated from LSCS.
线状硬皮病是一种局限性硬皮病，其特征为硬化病变呈线性、带状分布。线状硬皮病的“砍头刀样”亚型更常伴有全身发病，包括眼部、口腔和神经系统异常。在此，我们报告一例典型的线状硬皮病“砍头刀样”（LSCS）。最初，他表现为难治性部分性癫痫发作，然后才在头部出现特征性皮肤病变。这是一例罕见的明显脑实质受累的病例。我们没有开处方，而是对脑实质内病变进行了连续的脑磁共振成像随访。皮肤、面部和大脑的萎缩性改变仍然相同，在最近的随访中，他的癫痫发作也没有恶化。Parry-Romberg 综合征是一种非常相似的疾病，应与 LSCS 相鉴别。
