title: [Keratosis follicularis spinulosa decalvans].
abstract: Keratosis follicularis spinulosa decalvans is a rare, X-linked disorder of keratinization of the hair follicle with inflammation and atrophy associated with corneal dystrophy and other symptoms. A family with several affected members is reported. The unaffected parents were related. A 12-year-old girl and her 5-year-old brother had follicular spiny hyperkeratoses on the trunk and extremities. The girl had thinning of the eyelashes and eyebrows as well as scarring alopecia of the scalp as additional features of the disease. Both the girl and her brother had corneal dystrophy and photophobia. Two sisters aged 8 and 10 years did not show similar skin or eye findings.
authors.affiliation: Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universität Essen.
authors.forename: D
authors.initials: D
authors.lastname: Helbig
authors.forename: S
authors.initials: S
authors.lastname: Grabbe
authors.forename: T
authors.initials: T
authors.lastname: Jansen
mesh_terms: D002648:Child; D002675:Child, Preschool; D007644:Darier Disease / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics; D003879:Dermatologic Agents / Q000627:therapeutic use; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D016896:Treatment Outcome
chemical_list: D003879:Dermatologic Agents; D005938:Glucocorticoids