Bilateral segmental neurofibromatosis with partial unilateral lentiginosis.

Abstract

A 73-year-old woman presented with numerous cutaneous neurofibromas on the trunk in a bilateral dermatomal distribution (T2-T12). She also had multiple lentigines scattered on the left side of the trunk, affecting the T2-T12 dermatomes. The diagnosis of bilateral segmental neurofibromatosis (NF) was made on the basis of the clinical features and distribution of the neurofibromas and an absence of family and systemic involvement. The pattern of lentigines was consistent with that of partial unilateral lentiginosis (PUL). Bilateral segmental NF is rare and 15 cases had been reported in the literature so far. This is the first reported case of a patient with bilateral neurofibromas and PUL.