Autosomal Syndromic Ichthyosis

Ichthyosis-hypotrichosis-sclerosing cholangitis syndrome

  • Beginning: Birth.
  • Clinical picture: Severe / Very severe.
  • Syndromic: Associated with hair disorders.

It is a type of ichthyosis with a prevalence of 1 in every 1,000,000 births.

Neonatal ichthyosis-sclerosing cholangitis (NISCH syndrome) is a very rare and complex syndromic ichthyosis, characterized by hypotrichosis of the scalp, scarring alopecia, ichthyosis, and sclerosing cholangitis.