PDF(Pubmed)
Abstract：
A 29-year-old woman presented with dark-colored raised lesions on both eyelids since early childhood. Ophthalmological examination revealed pigmented verrucous lesions on her upper and lower eyelids bilaterally. The patient had a history of generalized tonic-clonic seizures. Dermatological examination revealed hyperpigmented verrucous plaques arranged along lines of Blaschko on the neck, trunk, and arms. On the basis of these findings, the diagnosis of epidermal nevus syndrome (ENS) was made. She had surgery for debulking of the lesions. Histological analysis revealed hyperkeratosis with foci of parakeratosis, acanthosis, and papillomatosis, consistent with linear verrucous epidermal nevus. Postoperative residual lesions did not respond to oral acitretin therapy (10 mg/kg/day for 2 months). Systematized ENS can rarely cause linear verrucous nevi on the upper and lower eyelids on both sides. These patients should be investigated for accompanying systemic anomalies and followed for potential malignant transformation of the skin lesions.
摘要：
一名29岁的妇女从幼年开始在两个眼睑上出现深色凸起的病变。眼科检查显示，双侧上下眼睑有色素性疣状病变。该患者有全身性强直阵挛性癫痫发作史。皮肤病学检查发现沿着Blaschko线排列在颈部的色素性疣状斑块，树干,和手臂。根据这些发现,诊断为表皮痣综合征(ENS)。她接受了切除病灶的手术。组织学分析显示角化过度伴角化不全病灶，棘皮病,和乳头状瘤病,与线状疣状表皮痣一致。术后残留病变对口服阿维A治疗（10mg/kg/天，持续2个月）无反应。系统化的ENS很少会在两侧的上下眼睑上引起线性疣状痣。应检查这些患者是否伴有全身异常，并随访皮肤病变的潜在恶性转化。
