BMJ Case Reports 2018; doi:10.1136/bcr-2017-223942
  • Rare disease

Dentin dysplasia: diagnostic challenges

  1. Kathryn Durey1
  1. 1 Restorative Dentistry, Leeds Dental Institute, Leeds, West Yorkshire, UK
  2. 2 Oral Medicine, Leeds Dental Institute, Leeds, Leeds, UK
  1. Correspondence to Dr Ahmed Alhilou, A.Alhilou{at}
  • Accepted 23 May 2018
  • Published 11 June 2018


Dentin dysplasia(DD) is a rare autosomal dominant disorder associated with disturbance of the dentin. While the crowns appear clinically normal, on radiography, the pulp spaces appear partially or completely obliterated, with short blunted roots, and multiple periapical radiolucencies affecting the apparently sound teeth. Clinical signs include spontaneous abscess formation or increased tooth mobility which can lead to exfoliation. DD can therefore have a significant impact on the patient’s dentition, and treatment is often challenging. Shields’ classification of dentin disorders has been recently criticised for failing to consider differential variations and expressions of these disorders. This paper describes a case of a 23-year-old woman with previously undiagnosed DD, who presented with clinical and histological features belonging to several of these diseases, thus highlighting the potential diagnostic challenges faced with Shields’ classification.


  • Contributors HPB, KD and AJM assisted in the clinical management of this patient and critical proof-reading of this manuscript. AA provided treatment for this patient and is the main author of this manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Register for free content

The full text of all Editor's Choice articles and summaries of every article are free without registration

The full text of Images in ... articles are free to registered users

Only fellows can access the full text of case reports (apart from Editor's Choice) - become a fellow today, or encourage your institution to, so that together we can grow and develop this resource

Don't forget to sign up for content alerts so you keep up to date with all the case reports as they are published, and let us know what you think by commenting on the Editor's blog

Navigate This Article