Neurofibroma of the mandible in an adolescent with von Recklinghausen's disease.

Neurofibromatosis, as identified by yon Recklinghausen, is an autosomal dominant, neurocutaneous syndrome, characterized by multiple neurofibromas, cafe-au-lait spots, and iris Lisch nodules.1, 2 This disorder has also been referred to as the "Elephant Man" disease, and was the subject of a play and movie depicting the life of Joseph Merrick, who suffered severely from neurofibromatosis. 2 Other features which can be associated with von Recklinghausen’s neurofibromatosis are macrocephaly, short stature, seizures, hypertension, deafness, constipation, kyphoscoliosis, developmental and learning disorders, cosmetic disfigurement, and neurofibrosarcomas, among others. 3 This genetic disorder was noted as early as 1793, but credited to Frederick von Recklinghausen who, in 1882, described two cases of his own and several others from the literature. 4 Neurofibromatosis occurs in one of every 3,000 live births in the United States, with more than 80,000 cases reported in 1981.5 There are no ethnic or racial predilections for the disease. 2