Segmental Neurofibromatosis: Atypical Localisation

Abstract

Neurofibromatosis (NF) is a genetic disease leading pathological findings in skin, soft tissue, bone and nervous system by affecting neural crest cells. Due to its heterogeneity neurofibromatosis was divided into eight different subgroups (NF-1 NF-VIII) by Riccardi. Segmental neurofibromatosis (NF type V) is characterized by cutaneous neurofibromas and Caf -au-lait spots limited with a segment of dermatome. Here we report this case with numerous, painless cutaneous nodules showing extension from the shoulder to the dorsal aspect of the right hand, since it a rare case.