University of the Ryukyus, Graduate School of Medicine, Okinawa, Japan.
Gunma University, Graduate School of Medicine, Okinawa, Gunma, Japan.
J Am Acad Dermatol. 2020 Sep;83(3):847-853. doi: 10.1016/j.jaad.2019.08.024. Epub 2020 Feb 3.
Trichorrhexis invaginata, the main diagnostic feature of Netherton syndrome, is often difficult to detect, especially in adult patients.
We sought to describe a characteristic feature of hairs in Netherton syndrome using a polarized light microscope and the underlying histopathologic changes.
Hairs obtained from 8 patients with Netherton syndrome were observed under polarized light, and we evaluated the correlation between number of band-like patterns and disease severity.
Under polarized microscopy, the hair shafts of 8 patients showed a characteristic band-like pattern under polarized light that was not observed in healthy control individuals or patients with atopic dermatitis. This discontinuity of polarized light shows a band-like pattern in which the bands mostly ranged from 0.1 to 1.0 mm in width. The observed ratio of this finding was significantly higher than that of trichorrhexis invaginata observed under light microscopy, and patients with severe dermatitis tended to have a higher ratio than those with less severe dermatitis.
Comparative examination among other congenital ichthyoses was not performed.
A band-like pattern in hairs with polarized light microscopy can be seen in Netherton syndrome and may have potential utility as a diagnostic marker.
扭结性发内凹，即 Netherton 综合征的主要诊断特征，通常很难检测到，尤其是在成年患者中。
我们试图通过偏光显微镜和潜在的组织病理学变化来描述 Netherton 综合征中毛发的一个特征性特征。
观察 8 例 Netherton 综合征患者的毛发在偏光显微镜下的表现，并评估带纹数量与疾病严重程度之间的相关性。
在偏光显微镜下，8 例患者的毛发显示出一种特征性的条带状模式，在健康对照组和特应性皮炎患者中均未观察到。这种偏振光的不连续性呈条带状，条带的宽度大多在 0.1 至 1.0 毫米之间。观察到的这种现象的比例明显高于光镜下观察到的扭结性发内凹，且皮炎严重程度较高的患者比皮炎较轻的患者出现该现象的比例更高。
未对其他先天性鱼鳞病进行对比检查。
偏光显微镜下的毛发条带模式可见于 Netherton 综合征，可能具有作为诊断标志物的潜在用途。
