Diseases with congenital or hereditary lesions of the skin and nervous system are often considered together as neurocutaneous disorders. The last thorough review of these conditions was Gomez' 1987 book Neurocutaneous Diseases. Since then, our knowledge of neurocutaneous disorders has increased dramatically. Detailed information about the natural history and clinical features has clarified many long-standing clinical riddles, and new information about the genetics of these conditions has provided insight into their classification, pathophysiology, and molecular biology.

The concept of neurocutaneous disorders unifies a group of rare neurological disorders whose initial identification depends on simple visual diagnosis. While each of the conditions included in this book is unique, the clinical utility of using cutaneous signs to help identify unusual genetic or congenital disorders is well established. Our aim is to provide readily accessible information about the clinical features and natural history of these rare conditions as well as an understanding of their genetic basis and molecular mechanisms.

We thank the colleagues who provided clinical photographs, radiographs, information, and encouragement. We are also indebted to the medical students, residents, and colleagues whose enthusiasm and eagerness to learn about neurocutaneous syndromes spurred us to complete this book.