Incontinentia pigmenti (IP), or Bloch-Sulzberger syndrome, is an X-linked dominant disease mainly of females that is lethal in males, caused by a mutation in the IKBKG gene. The disease is noticed at birth or within the first few weeks of life. The disease typically progresses through 4 stages: vesicular, verrucous, hyperpigmentation, and hypopigmentation / atrophy. Most cases are associated with developmental abnormalities of the skin, hair, nails, teeth, eyes, and central nervous system. Intellectual disability and epilepsy can be associated. Ocular findings, if present, include both retinal and nonretinal abnormalities, usually related to vascular occlusion.