Incontinentia pigmenti with ocular involvement: two cases

Genet Couns. 2012;23(1):57-63.


Incontinentia pigmenti (IP) is a rare, X-linked dominant disorder that presents at or soon after birth and presents with cutaneous signs and symptoms. Besides its typical skin signs presenting in all patients, central nervous system (CNS), ophthalmologic and dental involvement are encountered as well. In this report, we present two patients at the verrucous stage of IP and review the literature concerning the diagnosis and course of IP.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Eye / pathology*
  • Female
  • Humans
  • Incontinentia Pigmenti / diagnosis*
  • Infant
  • Skin / pathology*