Leukonychia totalis associated with multiple pilar cysts: report of a five-generation family: FLOTCH syndrome?

被引:7
作者
Rodriguez-Lojo, Romina [1 ]
Del Pozo, Jesus [1 ]
Sacristan, Felipe [2 ]
Barja, Juan [1 ]
Pineyro-Molina, Francisca [1 ]
Perez-Varela, Lucia [1 ]
机构
[1] CHU A Coruna, Dept Dermatol, La Coruna 15001, Spain
[2] CHU A Coruna, Dept Pathol, La Coruna 15006, Spain
关键词
leukonychia; pilar cysts; nail; FLOTCH syndrome; hair; Bauer syndrome; SEBACEOUS CYSTS; CONGENITAL LEUKONYCHIA; PARTIALIS;
D O I
10.1684/ejd.2011.1369
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
The association of familial totalis leukonychia with multiple pilar cysts is a rare condition that could represent a separate syndromic entity. Since Bauer described a family with totalis leukonychia and sebaceous cysts in 1920, only four new affected families have been reported. We report a five-generation family with a total leukonychia and multiple pilar cysts on the scalp. The hypothesis of a deficiency of a gene regulating the structure of keratin has been postulated but the exact genetic mechanism has not been yet determined. In our family, no other keratinizing structures were involved.
引用
收藏
页码:484 / 486
页数:3
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