Hypomelanosis of ito syndrome (incontinentia pigmenti achromians; systematized achromic nevus)

General: Probable autosomal dominant transmission; cutaneous abnormality consisting of bizarre, pattern 727h73h ed, macular hypopigmentation over variable portions of the body with multiple associated defects in other body systems; abnormal chromosome constitutions.

Ocular: Iridal heterochromia; myopia; esotropia; microphthalmia; hypertelorism; nystagmus; strabismus; corneal opacity; choroidal atrophy; exotropia; small optic nerve; hypopigmentation of the fundus; corneal asymmetry; pannus; atrophic irides with irregular pupillary margins; cataract; retinal detachment.

Clinical: Cutaneous manifestations consisting of macular hypopigmented whorls, streaks, and patches in a bilateral or unilateral distribution affecting almost any portion of the body surface; 50% have associated noncutaneous abnormalities, including central nervous system dysfunction (seizure, delayed development) and musculoskeletal anomalies.

Ito M. Studies of melanin XI. Incontinentia pigmenti achromians: a singular case of nevus depigmentosus systematicus bilateralis. Tohoku J Esp Med 1952; 55[Supp1]:57.

Schwartz MF, et al. Hypomelanosis of Ito (incontinentia pigmenti achromians): a neurocutaneous syndrome. J Pediatr 1977; 90:23-240.

Sybert VP. Hypomelanosis of Ito: a description, not a diagnosis. J Invest Dermatol 1994; 103[5 Supp1]:141S-l43S.

Weaver RG Jr, et al. The ocular changes of incontinentia pigmenti achromians (hypomelanosis of Ito). J Pediatr Ophthalmol Strabismus 1991; 28: 160-l63.