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.