一例Netherton综合征患者皮肤和毛发的电镜观察

目的：电镜观察Netherton综合征皮肤和毛发的超微结构改变。方法：对一例21岁Netllerton综合征男性患者的皮肤及头发毛干进行透射电镜及扫描电镜观察。结果：透射电镜观察显示，表皮角质层角质细胞分离，角质细胞间隙见板层小体、电子致密颗粒及变性桥粒，角质细胞内见多量脂滴。扫描电镜观察显示毛干呈竹节样改变伴有毛小皮断裂和剥脱。结论：表皮角质层细胞间的板层小体及电子致密颗粒是Netherton综合征特征性的超微结构。电镜观察对Netherton综合征的早期诊断有意义。

Electron microscopic observation of skin and hair on a case of Netherton syndrome

Objective: To observe the ultrastructural changes of skin and hair on a case of Netherton syndrome.Methods:The skin biopsy and hair shaft specimens were obtained from skin and hair of case of a 21-year-old male patient with Netherton syndrome and observed by TEM and SEM respectively.Results:TEM observation showed that the epidermis stratum corneum were characterized by the appearance of lamellar bodies,electron-dense granules, and degenerating desmosomes in the intercellular spaces with separation of corneocytes.The intracellular lipid droplets were seen.SEM observation revealed bamboo-like hair with broken cuticles of hair-shaft.Conclusion:The lamellar bodies and electron-dense granules in the intercellular spaces of stratum corneum are characteristic ultrastructural changes of the skin of Netherton syndrome.The ultrastructural observation is useful for the early diagnosis of Netherton syndrome.