title: Keratosis follicularis spinulosa decalvans: report of a case with ultrastructural study and unsuccessful trial of retinoids.
abstract: Keratosis follicularis spinulosa decalvans (KFSD) is a genetic disorder characterized by disseminated follicular hyperkeratosis, especially localizated to scalp and face. We report the case of a new patient displaying typical features of KFSD. Ultrastructural study was performed and displayed round keratohyalin granules in follicular keratinocytes. Trial with etretinate, which has not been reported before in this disease, proved to be ineffective.
authors.affiliation: Centre d'étude et de traitement des maladies génétiques à expression cutanée, Hôpital Saint Louis, Paris, France.
authors.forename: D
authors.initials: D
authors.lastname: Puppin
authors.forename: S
authors.initials: S
authors.lastname: Aractingi
authors.forename: L
authors.initials: L
authors.lastname: Dubertret
authors.forename: C
authors.initials: C
authors.lastname: Blanchet-Bardon
mesh_terms: D001706:Biopsy; D007644:Darier Disease / Q000151:congenital / Q000188:drug therapy* / Q000473:pathology; D005050:Etretinate / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D008297:Male; D008854:Microscopy, Electron; D012535:Scalp / Q000473:pathology / Q000648:ultrastructure*; D012536:Scalp Dermatoses / Q000151:congenital / Q000188:drug therapy* / Q000473:pathology; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D005050:Etretinate