Zabawski E J, Cohen J B
Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, USA.
Dermatol Online J. 1999 May;5(1):3.
The tooth-and-nail syndrome (Witkop's syndrome) is a rare autosomal dominant ectodermal dysplasia manifest by defects of the nail plates of the fingers and toes and hypodontia with normal hair and sweat gland function. We report a thirteen year-old girl who presented with marked longitudinal ridging of the nail plates of all ten fingers. The toenails were mildly ridged with koilonychia. Her mother's fingers were similarly affected to a lesser degree while her toenails appeared normal. Examination of the child's dentition revealed a hyperplastic frenulum and the absence of one of the usual four mandibular incisors. History provided by the mother described the maternal grandmother and maternal great aunt as having identical nail findings and the presence of only three lower incisors. Hair examination was normal in the mother and child, and no history or findings of sweat gland dysfunction was present. This report describes familial hypodontia, fingernail onychorrhexis, and toenail koilonychia consistent with Witkop's syndrome.
齿甲综合征（维特科普综合征）是一种罕见的常染色体显性外胚层发育不良，表现为手指和脚趾甲甲板缺陷以及牙发育不全，而毛发和汗腺功能正常。我们报告一名13岁女孩，其所有10个手指的甲甲板均有明显的纵向嵴。脚趾甲有轻度嵴伴匙状甲。她母亲的手指有类似但程度较轻的受累，而她的脚趾甲外观正常。检查该儿童的牙列发现有增生的系带，且通常的4颗下颌切牙缺1颗。母亲提供的病史表明，外祖母和姨祖母有相同的指甲表现，且只有3颗下切牙。母亲和孩子的毛发检查正常，也没有汗腺功能障碍的病史或检查结果。本报告描述了与维特科普综合征相符的家族性牙发育不全、手指甲纵裂和脚趾甲匙状甲。
