Destructive encephalopathy in incontinentia pigmenti: a primary disorder?

A 3-day-old infant with incontinentia pigmenti presented with acute encephalopathy associated with neuroradiographic findings of hemorrhagic necrosis and brain edema. No specific infectious, inflammatory, vascular, or metabolic abnormality was identified. We speculate that there is a variably expressed mutant protein in incontinentia pigmenti that could cause either developmental brain malformations or a destructive process.