Cutaneous Segmental Neurofibromatosis

A recent case of cutaneous segmental neurofibromatosis provided the opportunity to review the literature to better understand this rare disease. This cutaneous form of the disease usually appears only on the skin, without other systemic involvement; it is not inherited. Only 19 additional cases in the English literature could be found, three cases with extracutaneous manifestations; three cases were familial. The prognosis for this form of neurofibromatosis is excellent, but examination for evidence of systemic disease is indicated.