Hypomelanosis of Ito (incontinentia pigmenti achromians): a neurocutaneous syndrome

J Pediatr. 1977 Feb;90(2):236-40. doi: 10.1016/s0022-3476(77)80636-7.


Hypomelanosis of Ito (incontinentia pigment achromians, systematized achromic nevus) is a cutaneous abnormality consisting of bizarre, patterned, macular hypopigmentation over variable portions of the body surface. Multiple associated defects in other systems occur in a significant precentage of affected individuals. Most commonly, the central nervous system, eye, and musculoskeletal structures are involved. It is suggested that the cutaneous abnormality, which is often detectable at birth or during infancy, may forewarn pediatricians of the possible emergence of defects in other organ systems.

MeSH terms

  • Abnormalities, Multiple / diagnosis*
  • Child
  • Child, Preschool
  • Eye Diseases / complications
  • Female
  • Humans
  • Infant
  • Male
  • Nevus, Pigmented / complications
  • Nevus, Pigmented / diagnosis
  • Pigmentation Disorders / complications
  • Pigmentation Disorders / diagnosis*
  • Scoliosis / complications
  • Seizures / complications
  • Skin Neoplasms / complications
  • Skin Neoplasms / diagnosis
  • Syndrome