The Multiple Lentigines Syndrome

Melanocytes are derived in the embryo from the neuroectoderm. They are responsible in part for formation of the inner ear. There is a frequent association of congenital pigmentary abnormalities with hearing loss and neurologic diseases. Examination of patients with inherited pigmentary disorders might reveal that the genetic defect affects all tissues of neuroectodermal origin. The findings in our patient are consistent with this hypothesis. Electron microscopic findings of the lentigines are reported.