Hypomelanosis of Ito with neurological involvement

Iti Varshney, Mohammad Adil, Syed Suhail Amin, Mohd Mohtashim, Roopal Bansal, Hania Qamar


Hypomelanosis of Ito is also known as Incontinentia pigmenti achromians because the distribution of depigmented lesions is the negative image of hyperpigmented streaks of Incontinentia pigmenti. We report an infant of 9 months with hypopigmented macules along blaschkoid lines, developmental delay, macrocephaly and seizures. MRI brain revealed white matter atrophy and prominent temporal horns of lateral ventricles. This case report highlights the coexistence of Hypomelanosis of Ito with neurological involvement and thus the need to differentiate it from other similar conditions presenting with Blaschkoid dyspigmentation.



Hypomelanosis of Ito, Incontinentia Pigmenti Achromicans, neurocutaneous syndrome, white matter atrophy

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