Rigante Donato, Battaglia Domenica, Contaldo Ilaria, La Torraca Ilaria, Avallone Laura, Gaspari Stefania, Bersani Giulia, Stabile Achille
Center of Periodic Fevers, Department of Pediatric Sciences, Università Cattolica Sacro Cuore, Largo A. Gemelli no. 8, 00168, Rome, Italy.
Rheumatol Int. 2008 Jul;28(9):925-9. doi: 10.1007/s00296-008-0541-8. Epub 2008 Feb 16.
We report a 7-year-old boy who unexpectedly developed a multi-drug resistant epilepsy with negative neuroimaging results, followed by the insidious appearance of linear localized scleroderma involving the right leg. When the boy was 16 and severely affected by epileptic encephalopathy, we have evaluated this case for the first time: his localized scleroderma had reached the right buttock and positive anti-nuclear antibody was the only positive laboratory test. Methotrexate administered for 12 months was ineffective in improving both the organization of his electroencephalographic pattern and seizure control, though seemed to stabilize the progression of linear scleroderma. This report suggests that neurological abnormality and extracranial scleroderma might represent two own distinct processes in a same patient.
我们报告了一名7岁男孩，他意外地患上了多药耐药性癫痫，神经影像学检查结果为阴性，随后右腿出现了隐匿性线状局限性硬皮病。当该男孩16岁且严重受癫痫性脑病影响时，我们首次对该病例进行了评估：他的局限性硬皮病已蔓延至右臀部，唯一呈阳性的实验室检查是抗核抗体。给予甲氨蝶呤治疗12个月，在改善其脑电图模式和控制癫痫发作方面均无效，不过似乎稳定了线状硬皮病的进展。本报告表明，神经异常和颅外硬皮病可能是同一患者中两个不同的过程。
