SEGMENTAL NEUROFIBROMATOSIS - CASE-REPORTS AND REVIEW

Segmental neurofibromatosis (neurofibromatosis type V) is a rare disor der characterized by cafe-au-lait macules and neurofibromas, or only n eurofibromas, limited to one region of the body. Three patients with s egmental neurofibromatosis are described, and cases of this condition in the world literature are reviewed. Segmental neurofibromatosis has only been described in 82 patients, including our three, The median ag e at onset was 28 years and the incidence was higher in women (58%). T he neurofibromas most commonly occupied either a cervical or thoracic dermatome and were unilateral, occurring more often on the right side (43 patients) than the left (34 patients). Cafe-au-lait macules were p resent in 26% of patients. Axillary freckling was described in only ni ne patients, Disease-associated systemic involvement was uncommon. Mos t patients with segmental neurofibromatosis (93%) do not have a family history of neurofibromatosis.