Ichthyosis cribriformis: A new entity?

J Am Acad Dermatol. 2008 Mar;58(3):505-7. doi: 10.1016/j.jaad.2007.03.034.

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."

Publication types

  • Case Reports

MeSH terms

  • Administration, Oral
  • Adult
  • Dermatologic Agents / administration & dosage
  • Dermatologic Agents / therapeutic use
  • Dose-Response Relationship, Drug
  • Drug Administration Schedule
  • Female
  • Filaggrin Proteins
  • Humans
  • Ichthyosis / classification*
  • Ichthyosis / complications
  • Ichthyosis / pathology*
  • Isotretinoin / administration & dosage
  • Isotretinoin / therapeutic use
  • Keratoderma, Palmoplantar, Epidermolytic / drug therapy
  • Keratoderma, Palmoplantar, Epidermolytic / etiology*
  • Keratoderma, Palmoplantar, Epidermolytic / pathology*
  • Microscopy, Electron
  • Terminology as Topic
  • Treatment Outcome

Substances

  • Dermatologic Agents
  • FLG protein, human
  • Filaggrin Proteins
  • Isotretinoin