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Nevus of Jadassohn in a 5-year-old boy

(A) Face of a 5-year-old boy with a typical nevus of Jadassohn in the frontal region, dorsum of the nose and neck on the left side. A continuous line extends from the forehead onto the nose. He has epilepsy and mild intellectual deficit. (B) Axial MRI shows mild right hemimegalencephaly and subcortical laminar heterotopia of gray matter. (C) Sagittal MRI of same patient; cortical gyration is abnormal with both polymicrogyria occipitally and pachygyria frontally. An expansion of the frontal diploic space may be seen. (Contributed by Dr. Laura Flores-Sarnat.)

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Associated Disorders

  • Cardiac anomalies
  • Hemimegalencephaly
  • Infantile spasms
  • Ocular anomalies
  • Vitamin D-resistant rickets