title: Ichthyosis cribriformis: A new entity?
abstract: A 22-year-old woman had an unusual congenital skin disorder in the form of ichthyosis characterized by innumerable keratotic plugs, resulting in a peculiar sievelike appearance. On histopathologic examination the epidermis showed a pronounced orthohyperkeratosis. The keratotic plugs seemed to emerge from the infundibular region of hair follicles. Immunohistochemical characterization revealed a normal keratin and filaggrin expression. Oral treatment with isotretinoin resulted in a reduction of the hyperkeratosis. Because we could not find any previously reported similar case, we propose the new name "ichthyosis cribriformis," which means "sievelike ichthyosis."
authors.affiliation: Catholic and Federal University of Pelotas, Pelotas, Brazil. hiramalmeidajr@hotmail.com
authors.forename: Hiram
authors.initials: H
authors.lastname: Larangeira de Almeida
authors.forename: Rudolf
authors.initials: R
authors.lastname: Happle
authors.forename: Ulrike
authors.initials: U
authors.lastname: Blume-Peytavi
authors.forename: Luis Antônio Suita
authors.initials: LA
authors.lastname: de Castro
mesh_terms: D000284:Administration, Oral; D000328:Adult; D003879:Dermatologic Agents / Q000008:administration & dosage / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D000091344:Filaggrin Proteins; D006801:Humans; D007057:Ichthyosis / Q000145:classification* / Q000150:complications / Q000473:pathology*; D015474:Isotretinoin / Q000008:administration & dosage / Q000627:therapeutic use; D053546:Keratoderma, Palmoplantar, Epidermolytic / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D008854:Microscopy, Electron; D009626:Terminology as Topic; D016896:Treatment Outcome
chemical_list: D003879:Dermatologic Agents; C000717249:FLG protein, human; D000091344:Filaggrin Proteins; D015474:Isotretinoin