Neurofibromatosis type 1 (NF1) is the most common and well-understood neurodermatologic disease, with an incidence of approximately 1/3000 to 1 in 4000 individuals. NF1 is an autosomal dominant disease caused by mutations in the NF1 gene located on chromosome 17q11.2. Approximately 50% of NF1 cases are familial, and the remaining cases occur sporadically.

Neurofibromatosis type 2 (NF2) is an autosomal dominant multisystem disease that often leads to the occurrence of multiple benign neuroendocrine tumors, central nervous system tumors, eye abnormalities, and nerve-skin lesions.