During the past years, our knowledge on genodermatoses has undergone dramatic changes. In the light of the present molecular research, the vehement discussions regarding nosological categories that prevailed in the past century are now coming to an end, and new avenues of thinking have been opened.Classically, the term genodermatoses is applied to the relatively rare single gene disorders.Genodermatoses may affect epidermal, dermal or specialized structures.Skin & oral cavity are akin to each other in several attributes; both are made up of stratified squamous cells, are keratinized & have ectodermal origin; hence frequently genodermatoses demonstrate oral involvement & in several conditions, the oral presentation is distinct, giving first inkling of an underlying genetic skin disorder

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