Abnormal Nevoblast Migration Mimicking Neurofibromatosis

A patient appeared to have von Recklinghausen type I neurofibromatosis, but her numerous cutaneous tumors were intradermal nevi and not neurofibromas. The patient had hundreds of 1- to 3-cm firm, flesh-colored, dome-shaped papules and pedunculated nodules on her buccal mucosa, eyelids, face, extremities, and trunk as well as a large, confluent, cerebriform tumor extending from the 12th thoracic vertebra to the sacrum. No cafe au lait macules, freckles, or Lisch nodules were present. Several hundred lesions were removed using the carbon dioxide laser. Histopathologic examination of all of the lesions revealed all of them to be intradermal nevi. Our patient's skin disease was clinically very similar to neurofibromatosis. We suggest our patient represents a distinct clinical entity that is related to environmental factors or a mutation that affected nevoblasts or melanoblasts and their derivatives during early embryo development.