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Hypomelanosis of Ito (incontinentia pigmenti achromians)—a clinicopathologic study

Macrocephaly and gray matter heterotopias

Department of Neurology (Dr. Ross), Cincinnati Center for Developmental Disorders, and the Departments of Pathology (Neuropathology) and Surgery (Neurosurgery) (Dr. Liwnicz), University of Cincinnati, OH, and Division of Pediatric Neurology (Dr. Chun), and the Department of Pathology and Pediatrics (Dr. Gilbert), Medical College of Wisconsin, Madison, WI.

We studied a boy with macrocephaly, hypotonia, pigmentary retinopathy, unilateral whorled hypopigmented skin lesions, and seizures. Skin biopsy confirmed the clinical diagnosis of hypomelanosis of Ito. Postmortem examination at age 22 months revealed a severe neuronal migrational defect that altered the cerebral cortex architecture of white matter. There were many gray matter heterotopias characterized by altered neurons and giant cells. Electronmicroscopy revealed the astrocytic nature of the giant cells. Embryologic migration of both melanoblasts from neural crest and cortical neurons occurs in the second trimester, suggesting a common mechanism for the developmental pathology of skin and brain.

Address correspondence and reprint requests to Dr. Ross, Cincinnati Neurologic Assoc., Inc., 111 Wellington Place, Cincinnati, OH 45219.

Accepted for publication February 24, 1982.

This article has been cited by other articles:

				M. Ruggieri and L. Pavone Topical Review: Hypomelanosis of Ito: Clinical Syndrome or Just Phenotype? J Child Neurol, October 1, 2000; 15(10): 635 - 644. [Abstract] [PDF]