X-linked Ichthyosis: Relation Between Cholesterol Sulphate, Dehydroepiandrosterone Sulphate and Patient's Age

Br J Dermatol. 1998 Apr;138(4):655-7. doi: 10.1046/j.1365-2133.1998.02180.x.

Abstract

Steroid sulphatase deficiency is a feature of recessive X-linked ichthyosis (RXLI) that causes the accumulation of sulphated steroids (SS) in various organs and cells. In a previous study, we detected elevated cholesterol sulphate (CS) and dehydroepiandrosterone sulphate (DHEAS) serum levels in a group of 15 RXLI patients selected in a narrow age range. In the present study both CS and DHEAS serum levels were qualitatively and quantitatively determined using gas-chromatographic analysis in a group of 33 RXLI patients ranging in age from 3 to 70 years. The levels of CS and DHEAS were significantly increased in all patients. Variations in SS were related both to patients' ages and clinical course of the disease; Serum SS levels start to increase in early infancy, peak at puberty, remain elevated in adults and decrease slightly in the elderly.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Aged
  • Child
  • Child, Preschool
  • Cholesterol Esters / blood*
  • Chromatography, Gas
  • Dehydroepiandrosterone Sulfate / blood*
  • Humans
  • Ichthyosis, X-Linked / blood*
  • Male
  • Middle Aged

Substances

  • Cholesterol Esters
  • Dehydroepiandrosterone Sulfate
  • cholesteryl sulfate