[The genetics and molecular genetics of X-chromosomal recessive ichthyosis]

X-linked ichthyosis has been shown to be associated with the deficiency of the steroid sulfatase/arylsulfatase C. The molecular biological results are reviewed concerning the localization of the steroid sulfatase gene to the distal short arm of the X chromosome and the molecular defects of this gene in patients of X-linked ichthyosis. The conclusions are summarized for genetic counselling, carrier detection and prenatal diagnosis.