Neurocutaneous syndromes associated with pigmentary skin lesions

Tremendous strides have been made in our understanding of genetic disorders, including those that involve both the skin and the nervous system. The list of well-established neurocutaneous syndromes has increased during a few decades from a few classical phakomatoses to more than 30 entities. The dermatologist has the opportunity to facilitate precise diagnosis and optimal care for these patients by recognition of the cutaneous markers for these diseases. We have attempted to provide an overview of some recent advances in those syndromes manifested by pigmentary skin changes, emphasizing the clinical spectrum of each disorder and providing guidelines for an approach to management.