成人表皮痣综合征1例

 报告1例成人表皮痣综合征。患者男，28岁，因皮疹、智力低下、多动28年,间断抽搐27年就诊。皮肤科检查：额部、眼周、颈部可见弥漫性密集的淡褐至褐黑色乳头瘤样角化性丘疹，触之坚硬，似高起鱼鳞病、先天性良性黑棘皮病样。躯干、双上肢、四肢皮损角质增厚，较额部、眼周、颈部薄，可见弧形带状或旋涡状排列,不规则斑片状或斑点状色素脱失斑，与增生性皮损及正常皮肤相互交错。颈部皮损组织病理检查示纤维上皮性息肉改变，胸部皮损组织病理检查示表皮痣样改变。脑电图：脑电图异常伴高中波幅尖波及4~7 Hz的θ节律短阵出现。诊断为表皮痣综合征。

A case of epidermal nevus syndrome

 A 28-year old male was diagnosed with rash, mental retardation, hyperactivity disorder for 28 years, and intermittent convulsions for 27 years. Diffuse and dense pale brown to brown black papilloma like keratinizing papules could be seen on the forehead, around the eyes, and neck, which were hard to touch, like high ichthyosis and congenital benign acanthosis nigricans. The skin cutin of skin lesions of trunk, upper limbs and limbs was thickened, thinner than that of forehead, periocular and neck. Arc banded or vortex arrangement, irregular patchy or spotted depigmentation spots can be seen, which are staggered with proliferative skin lesions and normal skin. Histopathological results showed fibroepithelial polyp changes and epidermal nevus like changes. Abnormal EEG with high and medium amplitude tip and 4~7 hz θ short rhythmic array appeared. The patient was diagnosed with epidermal nerus syndrome.