ABSTRACT
Incontinentia pigmenti is an uncommon genodermatosis presented by characteristic cutaneous stages and frequent association with one or more ectodermal or mesodermal anomalies. A new-born girl, 2 days old, had had crops of erythematous papules and vesicles on the trunk and extremities. At 2 years of age, she had the whorled hyperpigmentation. There were no anomalous features in teeth, eyes and hair, but she had a neurologic disorder. Left hemiplegia, related to upper motor neuron weakness, showed brisk left DTR, spastic Achilles tendon with walking disorders, decreased grasping power of left hand and weakness of left upper extremity. Brain MRI revealed marked loss of volume in the white matter of the right lateral ventricle in T1 weighted images. We report herein an explicit case of incontinentia pigmenti associated with right cerebral hemiatrophy.
