A 2 1/2-year-old girl with hypomelanosis of Itô is described. There is a bizarre distribution of congenital depigmentation stopping abruptly at the midline anteriorly, with whorl-like and zig-zag patterning. As can happen in this syndrome, there is severe retardation and intractible epilepsy, with computerized tomography showing gross cerebral atrophy.
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