Introduction: Some cutaneous lesions might be the first clinical manifestation of diseases with systemic involvement. This article aims to review the relevant dermatological semiology for the concerning diseases with neurological involvement in childhood.
Material and Methods: Review of the related literature indexed to MedLine published in the last twelve years.
Results: The main relevant nosologic groups include the genodermatosis (namely neurocutaneous syndromes), pigmentary, vascular and endocrine disorders, congenital enzymatic deficiencies and occult spinal dysraphisms.
Discussion: The recognition of specific cutaneous signs is important once may enable a much earlier diagnosis.
Keywords: Child; Nervous System Diseases; Neurocutaneous Syndromes; Skin Diseases.

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