Segmental Neurofibromatosis with Angioedema on the Same Side of Face: Report of an Interesting Case
Author(s): Shikhar Ganjoo*, MPS Sawhney, Uma Sharma and Tulika Gupta
Abstract
Neurofibromatosis (NF) is a genetic disorder arising from neural crest derived cells. Riccardi suggested a classification for NF dividing it into eight subtypes with segmental neurofibromatosis (SNF) being type 5. SNF is characterized by neurofibromas, café-au-lait macules (CALM) or only neurofibromas distributed in a particular segment of the body. We describe one patient who showed segmental expression of the disease along with angioedema on the same side of face. Cutaneous neurofibromas arise from small peripheral nerves and are composed of Schwann cells, fibroblasts, degranulation mast cells, and vascular cells. Increased number of mast cells in neurofibromatosis may be responsible for pruritus. Such patients may also have localized angioedema due to degranulation of this increased number of mast cells.