4 – Neurokutane Erkrankungen

The term “neurocutaneous disorders” refers to a heterogeneous group of congenital, mostly inherited diseases manifesting in tissues of neuroectodermal origin. Because of their aetiology a causative therapy is not possible at present. Nevertheless, new therapeutic options are emerging as a result of a growing knowledge about the signal cascades involved in the diseases as well as the interdisciplinary medical care of the patients and individualized, often quite aggressive therapeutic regimens. The application of anti-angiogeneic substances against vestibular schwannoma in neurofibromatosis type 2 and the treatment with rapamycin in tuberous sclerosis are just two examples in this context. The chapter gives an overview on the clinic, diagnosis and current state of evidence based therapy of the more common neurocutaneous disorders.