Darier disease: a case report

Jalil, A.A.; Zain, R.B.; van der Waal, I. (2005) Darier disease: a case report. British Journal of Oral and Maxillofacial Surgery, 43 (4). pp. 336-338. ISSN 0266-4356

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      Darier disease, also known as keratosis follicularis, dyskeratosis follicularis, and benign dyskeratosis, is a rare disorder of keratinisation that primarily affects the skin and, to a lesser extent, the oral mucosa. It was described independently by both Darier and White in 1889. It has a prevalence of 1:100,000 of the population and is inherited as an autosomal dominant trait.1 The age of onset is childhood or adolescence. Patients usually present with multiple small firm reddish-brown papules on the forehead, scalp, neck, shoulders, chest, and limbs. Other cutaneous signs include punctate keratotic pits of the palms and soles and dystrophy of the nails, which is characterised by a red and white sandwich of streaks associated with a V-shaped notch. The oral lesions are usually asymptomatic and are seen as multiple, normal-coloured or white flat-topped papules that predominantly affect the palate.

      Item Type: Article
      1. Jalil, A.A.( Oral Medicine, Oral Pathology and Periodontology Department, Faculty of Dentistry, University of Malaya, Kuala Lumpur, Malaysia )
      2. Zain, R.B.(Department of Oral Pathology and Oral Medicine and Periodontology, Faculty of Dentistry, University of Malaya)
      3. van der Waal, I.(b Department of Oral and Maxillofacial Surgery and Pathology, ACTA, Vrije Universiteit Medical Centre, De Boelelaan 1117, 1007 MB Amsterdam, Netherlands)
      Journal or Publication Title: British Journal of Oral and Maxillofacial Surgery
      Additional Information: Prof. Dr. Rosnah Mohd Zain Department of Oral Pathology and Oral Medicine and Periodontology, Faculty of Dentistry, University of Malaya
      Uncontrolled Keywords: Benign Dyskeratosis, Darier Disease, Dyskeratosis Follicularis, Keratosis Follicularis, Oral Squamous Cell Carcinoma, OSCC, Lichenoid Lesions, Lichen Planus, Oral Cancer, Oral Tumours, Pemphigus, Traumatic Eosinophilic Pranuloma, Aphthous Ulcers, Oral Mucosal Lesions, Betel Chewers Mucosa, Betel Quid Related Lesions, Betel Quid, Areca Quid, Tobacco Quid, Oral Cancer Screening, Training and Calibration, Early Detection, Oral Cancer Awareness, Biobanking, Tissue Bank, Databank, Oral Cancer, Tissue Bank, Research Credibility, Research Ethics.
      Subjects: R Medicine
      R Medicine > RK Dentistry
      R Medicine > RK Dentistry > Oral surger
      Divisions: Faculty of Dentistry > Dept of Oral Pathology & Oral Medicine & Periodontology
      Depositing User: Prof. Dr. Rosnah Mohd Zain
      Date Deposited: 16 Jan 2012 10:17
      Last Modified: 20 Oct 2014 13:25
      URI: http://eprints.um.edu.my/id/eprint/2431

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