Dentin dysplasia is a rare, hereditary disorder affecting the dental hard tissue. It is a congenital, autosomal dominant disease of unknown etiology that affects 1:100,000 populations. It may present as such affecting only the dental hard tissue or as one of the symptoms of underlying diseases such as calcinosis, Ehlers-Danlos syndrome, rhematoid arthitis, Vitaminosis D and Branchioskeletogenital syndrome.
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