[Teeth and oral mucosa in neurocutaneous syndromes, metabolic diseases and in diseases with defects of DNA repair]

Oral mucosa is frequently involved in a group of genetic diseases, which affect the skin and other organs, particularly the central nervous system. The lesions may be of characterized by inflammations in neurocutaneous syndromes and by tumoral lesions in those diseases with defects of DNA repair. The teeth are mainly involved in progeria, while jaw keratocysts are highly characteristic in Gorlin's syndrome.