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手掌病变处颗粒层角蛋白病的皮纹墨测试。

Dermoscopic furrow ink test of the palmar lesion in loricrin keratoderma.

机构信息

Department of Dermatology, Kurume University School of Medicine, Kurume, Japan.

Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan.

出版信息

J Dermatol. 2022 Aug;49(8):783-786. doi: 10.1111/1346-8138.16405. Epub 2022 Apr 25.

DOI:10.1111/1346-8138.16405
PMID:35467044
Abstract

Palmoplantar keratodermas (PPK) comprise a heterogeneous group of keratinization disorders that gradually progress during childhood, resulting in difficulties to establish a diagnosis and to identify a candidate gene for sequencing. Dermoscopic examination with staining of palmoplantar skin using a whiteboard marker, so-called "furrow ink test", could be a useful tool for differentiation between furrow and ridge in understanding the morphological characteristics of PPK. One of the striking features in autosomal dominant loricrin keratoderma (LK) is diffuse PPK with honeycomb pattern. In this study, we performed dermoscopic furrow ink test in a Japanese family of LK with the most frequent mutation c.684dup, p.Ser229Valfs*107 in the loricrin gene. The severe lesion revealed that irregular circular hyperkeratoses were aggregated and normal structures of furrows and ridges were disrupted. To accurately describe the nature of this dermoscopic patterned skin surface, we suggest that the condition could be termed as "irregular cobblestone appearance" rather than "honeycomb pattern". Regular cobblestone appearance to maintain parallel furrow structure was observed in early or mild hyperkeratotic lesions. Eccrine sweat glands that open on the surface of ridges nearly disappeared, resulting in hypohidrosis.

摘要

掌跖角化病(PPK)是一组异质性的角化障碍,在儿童时期逐渐进展,导致诊断困难,并难以确定候选基因进行测序。使用白板笔对掌跖皮肤进行染色的皮肤镜检查,即所谓的“沟纹墨水试验”,可以作为区分沟纹和脊的有用工具,有助于了解 PPK 的形态特征。常染色体显性层粘连蛋白角蛋白病(LK)的一个显著特征是弥漫性 PPK 伴蜂窝状模式。在这项研究中,我们对携带层粘连蛋白基因突变 c.684dup,p.Ser229Valfs*107 的常染色体显性 LK 的日本家系进行了皮肤镜沟纹墨水试验。严重的病变显示,不规则的圆形过度角化聚集,并且破坏了正常的脊和沟结构。为了准确描述这种皮肤镜图案状皮肤表面的性质,我们建议将其称为“不规则鹅卵石外观”,而不是“蜂窝状模式”。在早期或轻度过度角化病变中,观察到规则的鹅卵石外观以维持平行的脊结构。在脊表面开口的外分泌汗腺几乎消失,导致少汗。

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