Leukoencephalopathy: Unusual sonographic finding in a neonate with incontinentia pigmenti

Incontinentia pigmenti (IP) is a rare X-linked dominant neurocutaneous syndrome chiefly involving the ectodermal tissues, such as the skin, teeth, hair, eyes, and central nervous system (CNS). The diagnosis is usually made when the pathognomonic skin lesions are present; these typically appear in 4 stages: Erythematous vesicular rash, verrucous patches, swirling hyperpigmentation, and atrophic scarring. The exact pathogenesis and timing of neurologic involvement are not well known. It often manifests in the neonatal period with seizures paralleling the eruption of the vesicular rash. White matter abnormalities have been reported in IP.2-6 However, to our best knowledge, the sonographic characterization of white matter involvement has rarely been described. We report a case of IP with hyperechogenicity of the white matter on cranial sonography. The aim of this report is to suggest that IP should be considered as one etiology of leukoencephalopathy in neonates with vesicular eruption.