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This disease has an incidence of 1 per 1000 new patients consulting a paediatric neurological service or 1 per 8000-10000 unselected patients in a childrens hospital. 712006 hypomelonosis of Ito. Hypomelanosis Of Ito Streaks And Whorls Bmj Case Reports Hypomelanosis of Ito also known as incontinentia pigmenti achromians is the third most frequent phakomatosis involving a wide spectrum of defects in multiple organ systems. Hypomelanosis of ito mri brain . A severe form of hypomelanosis of Ito in two-year-old white girl is reported. MR findings We present a patient with hypomelanosis of Ito who showed extensive white matter lesions on brain MRI. Hypomelanosis of Ito can occur in the neonate infancy or childhood be isolated or diffuse often following the Blaschko lines and can fade in childhood or adulthood. One patient had a medulloblastoma three had cortical malformations and five demonstrated minor abnormalities such as dilated Virchow-Robin spaces or brain atrophy. The pu