Patrizi A, Pizzino D, Tullini A, Gobbi G, Santucci M
G Ital Dermatol Venereol. 1989 Jan-Feb;124(1-2):55-8.
The case of a 7-year-old girl, born from a monochorionic biamniotic pregnancy (with healthy male twin) is presented. The patient showed a congenital brown-yellowish, raised, rough and oval-shaped nevic neoformation of the right temporo-zygomatic region. At 28 days of age she had a right hemiclonic status epilepticus, and from the 8th month of life she presented right sided partial motor seizures. Moreover from the beginning, her development milestones were delayed. When she was 5 years old an electroencephalogram displayed a sharp asymmetrical background activity, a continuous paroxysmal activity on the right hemisphere and independent focal irritative anomalies on the left posterior region. Her computed tomographic scan demonstrated megalencephaly on the right hemisphere. A biopsy specimen of her skin lesion showed the histological characteristics of a nevus sebaceous of Jadassohn. Taken together the clinical, neuroradiological and dermatological data led to the diagnosis of linear nevus sebaceous syndrome.
本文报告了一名7岁女童的病例，她出生于单绒毛膜双羊膜囊妊娠（其同卵双胞胎为健康男婴）。患者右侧颞颧部有一个先天性棕黄色、隆起、粗糙的椭圆形痣样新生物。28日龄时，她出现了右侧半侧阵挛性癫痫持续状态，自8月龄起出现右侧部分性运动性癫痫发作。此外，从一开始她的发育里程碑就延迟了。5岁时，脑电图显示背景活动尖锐不对称，右半球有持续的阵发性活动，左后区有独立的局灶性刺激性异常。她的计算机断层扫描显示右半球巨脑症。皮肤病变的活检标本显示了 Jadassohn 皮脂腺痣的组织学特征。综合临床、神经放射学和皮肤病学数据，诊断为线状皮脂腺痣综合征。
