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家族性匙状甲

Familial koilonychia.

作者信息

Gao X H, Li X, Zhao Y, Wang Y, Chen H D

机构信息

Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang 110001, China.

出版信息

Int J Dermatol. 2001 Apr;40(4):290-1. doi: 10.1046/j.1365-4362.2001.01212-5.x.

DOI:10.1046/j.1365-4362.2001.01212-5.x
PMID:11454091
Abstract

A 2-year-old boy with flat and spoon-shaped nail plates was referred to us. His mother had noticed that his fingernails were quite soft when he was born. After 2 months, the nails became flat or spoon-shaped; the toenail plates also became rough and flat. Systemic examination revealed nothing unusual. All his fingernail plates were quite thin, and had concave surfaces; the distal edges of some nail plates were rough and darkened (Fig. 1). His great, second and middle toenails on both sides showed the same abnormalities as the fingernails. Routine laboratory tests were normal; serum levels of iron, zinc, calcium, and magnesium were within normal limits. Repeated microscopic examination and culture of fungi from both fingernails and toenails were negative. Histologic examination of the distal edges of some of his fingernail plates was basically normal. When checking members of the family, we noticed that the boy's mother and maternal grandmother had similar nail problems (out of 14 members of three generations), although no systemic or other skin disorders were noticed. All the fingernails of the boy's mother were lusterless and concave with darkening of the distal edges (Fig. 2). All her toenails were rough, lusterless, and brown; some of them had longitudinal ridges. His maternal grandmother had light brown colored fingernail plates with concave profiles (Fig. 3), and dark brown or even blackish colored toenails. Both the mother and maternal grandmother acknowledged that their nails were abnormal from early childhood. Repeated microscopic examination and culture of fungi from both fingernails and toenails were negative. Several distal portions of the nail plates were collected for histologic examination. The fingernail plates of the boy's mother showed numerous parakeratotic cells in the middle zone, and those of his maternal grandmother showed diffuse parakeratotic cells; melanin granules were not found in those nail plates that were checked. Taken together, a diagnosis of familial koilonychia was established.

摘要

一名2岁男孩因指甲扁平呈匙状被转诊至我们这里。他的母亲注意到他出生时指甲就相当柔软。2个月后,指甲变得扁平或呈匙状;趾甲也变得粗糙且扁平。全身检查未发现异常。他所有的指甲板都很薄,表面呈凹陷状;一些指甲板的远端边缘粗糙且颜色变深(图1)。他双脚的拇趾、第二趾和中趾的指甲与手指甲表现出相同的异常。常规实验室检查正常;血清铁、锌、钙和镁水平均在正常范围内。对手指甲和趾甲反复进行真菌显微镜检查及培养均为阴性。对他一些指甲板的远端边缘进行组织学检查基本正常。在检查其家庭成员时,我们注意到男孩的母亲和外祖母有类似的指甲问题(三代共14名成员中),尽管未发现全身或其他皮肤疾病。男孩母亲的所有手指甲均无光泽且呈凹陷状,远端边缘颜色变深(图2)。她所有的趾甲都粗糙、无光泽且呈褐色;其中一些有纵嵴。他的外祖母手指甲板呈浅褐色,有凹陷外形(图3),趾甲呈深褐色甚至黑色。母亲和外祖母都承认她们的指甲自幼就不正常。对她们的手指甲和趾甲反复进行真菌显微镜检查及培养均为阴性。采集了几块指甲板的远端部分进行组织学检查。男孩母亲的指甲板在中间区域有大量不全角化细胞,他外祖母的指甲板有弥漫性不全角化细胞;在所检查的那些指甲板中未发现黑色素颗粒。综合考虑,诊断为家族性匙状甲。

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