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Images in Neurology
Aug 2011

Incontinentia Pigmenti: Skin and Magnetic Resonance Imaging Findings

Arch Neurol. 2011;68(8):1080. doi:10.1001/archneurol.2011.164

After an uncomplicated delivery, a newborn girl was found to have a rash consisting of semilinear rows of vesicles and hyperpigmented papules (Figure 1). The patient's neurological examination was otherwise unremarkable. Examination of the mother revealed faint hypopigmentation behind the left knee and abnormal dentition to include peg-shaped upper incisors. The patient had right-sided seizures on her fourth day of life, consisting of clonic asynchronous facial, arm, and leg twitching. Magnetic resonance imaging of the brain revealed multiple diffusion-restricted lesions in the deep and subcortical white matter (Figure 2), seen more in the left hemisphere than in the right. A clinical diagnosis of incontinentia pigmenti was made, and genetic counseling was provided; confirmatory genetic testing was declined by the mother.