ICHTHYOSIS LINEARIS CIRCUMFLEXA: NOT ALWAYS A PART OF NETHERTON SYNDROME

Genet Couns. 2016;27(3):353-356.

Abstract

Ichthyosis Linearis Circumfiexa (ILC) is a rarely seen autosomal recessive keratinization disorder and is characterized by erythematous, polycystic, plaques with 'double-edged' scales. Its histological features resemble psoriasis. A triad of ichthyosis (usually Ichthyosis Linearis Circumfiexa), trichorrhexis invaginata (a distinctive hair shaft disorder) and atopy is named as Netherton syndrome. Herein, we report a 12 year-old girl presenting with ILC not accompanied with typical atopy findings and hair shaft disorder.

Publication types

  • Case Reports

MeSH terms

  • Administration, Cutaneous
  • Biopsy
  • Child
  • Female
  • Humans
  • Ichthyosis, Lamellar / diagnosis*
  • Ichthyosis, Lamellar / drug therapy
  • Ichthyosis, Lamellar / genetics*
  • Ichthyosis, Lamellar / pathology
  • Netherton Syndrome / diagnosis*
  • Netherton Syndrome / drug therapy
  • Netherton Syndrome / genetics*
  • Netherton Syndrome / pathology
  • Petrolatum / administration & dosage
  • Salicylic Acid / administration & dosage
  • Skin / drug effects
  • Skin / pathology

Substances

  • Petrolatum
  • Salicylic Acid