Genomic instability is a feature of Xp22.31 region wherein deletions or mutations are associated with X-linked ichthyosis (OMIM 308100, XLI), a dermatologic disorder presenting with dry, scaly skin due to a deficiency of the enzyme steroid sulfatase (STS), usually arising from a mutation in the STS gene [2–4]. Here, STS is linked to syndromic recessive X-linked ichthyosis.