Abstract

Dentin dysplasia is a rare autosomal dominant disorder affecting dentin and resulting in early loss of teeth. Although dentin dysplasia type I is reported to have an incidence of 1 in 100,000, dentin dysplasia type II is considered to be an extremely rare genetic disorder, the prevalence of which is not clearly reported in the literature so far. Moreover, there remains a lot of controversy regarding classification, terminologies, treatment options, and prognosis of dentin dysplasia. We present here an exclusive report and management by full mouth rehabilitation of two cases of dentin dysplasia type II in siblings with some peculiar clinical characteristics, making it one of a kind of case reports documented so far.

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