Publication at Second Faculty of Medicine |
2018
Abstract
Neurocutaneous disorders is a group of congenital, genetically conditioned, multisystem diseases that primarily affect the skinand nervous system, and also other body systems. They arise from the abnormal neuroectodermal tissue in the wrong courseof neural criste development.
All neurocutaneous disorders are among the rare diseases. They manifest from the neonatal ageto adulthood and have very variable clinical course, with a slow progression.
An important feature is the frequent occurrence oftumor processes, in childhood primarily benign character, in adulthood the risk of malignant tumors increases. The most commonneurocutaneous disease is neurofibromatosis von Recklinghausen type 1 (NF1).