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报告一例Bjrnstad综合征。患儿女,11岁。出生后即有头发扭曲,听力障碍,并进行性加重。显微镜下头发部分变扁平,有多处狭窄。头皮组织病理检查:毛囊内毛发断面长轴偏离毛囊纵轴线,显示毛发在毛囊内已扭曲。部分毛囊纤维性毛根鞘增厚,毛囊及其周围有少量淋巴细胞浸润。扫描电镜下发干扭曲,致发干表面出现纵嵴和凹沟,部分毛小皮细胞卷曲、脱落,血清微量元素铜值水平正常。
Report a case of Björnstad syndrome. Children with children, 11 years old. Twisted hair, hearing loss and progressive increase after birth. Hair under the microscope part of the flat, there are many narrow. Scalp histopathology: the major axis of the hair follicle within the hair follicle deviates from the longitudinal axis of the hair follicle, indicating that the hair has been distorted within the hair follicle. Some hair follicles fibrous root sheath thickening, hair follicles and the surrounding a small amount of lymphocyte infiltration. Under the scanning electron microscope, the hair was distorted. The longitudinal ridges and valleys appeared on the dry surface, and some of the hairy skin cells curled and exfoliated. The serum trace element copper level was normal.