hypomelanosis of Ito

Last reviewed 01/2018

Incontinentia pigmenti achromians is a disorder characterised by a whorl-like distribution of hypopigmentations of the skin. The usual distribution is over the chest and abdomen. The areas of hypopigmentation are usually present from birth. Often there is involvement of the CNS and mental retardation and seizures are common manifestations.

This condition is the result of somatic mosaicism - two cell lines are present, one with a normal karyotype and one with an abnormal karyotype.