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Epidermal nevus syndrome in a 10-year-old boy

Face of a 10-year-old boy with a lightly pigmented keratinocytic nevus and a lipomatous mass on the right side of the face; he has right hemimegalencephaly and intellectual disability with left hemiparesis and left-sided epilepsia partialis continua. This case is an example of Heide's syndrome. (Contributed by Dr. Laura Flores-Sarnat.)

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Associated Disorders

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