JLE

European Journal of Dermatology

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Leukonychia totalis associated with multiple pilar cysts: report of a five-generation family: FLOTCH syndrome? Volume 21, numéro 4, July-August 2011

Auteurs
Department of Dermatology, CHU A Coruña, Sir John Moore s/n., 15001 A Coruña Spain, Department of Pathology, CHU A Coruña, As Xubias 84, 15006, A Coruña, Spain

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.