World Journal of Dentistry

Register      Login

VOLUME 2 , ISSUE 2 ( April-June, 2011 ) > List of Articles


Darier's Disease: A Clinical Rarity

Roopa S Rao, Vijaya V Mysorekar, TK Sumathy

Citation Information : Rao RS, Mysorekar VV, Sumathy T. Darier's Disease: A Clinical Rarity. World J Dent 2011; 2 (2):139-142.

DOI: 10.5005/jp-journals-10015-1071

Published Online: 01-09-2012

Copyright Statement:  Copyright © 2011; The Author(s).


Darier's disease (DD) is a rare autosomal dominant genodermatosis clinically manifested by greasy hyperkeratotic papules primarily affecting seborrheic areas with less frequent involvement of the oral mucosa. Oral manifestations, if present, are usually asymptomatic, and are discovered in routine dental examination. Acantholysis and dyskeratosis represented by corps ronds and corps grains are the typical histological findings.

We report a case with clinical signs of DD in the palatal mucosa and skin. Skin biopsy and analysis of clinical history helped to diagnose DD. We report this case because of its clinical rarity as the prevalence of the disease has been estimated at 1/50,000-1,00,000. This could be a case of sporadic mutation as there was no family history. This case emphasizes the importance of oral examination among dermatologists, clinicians and pathologists to clinch the diagnosis of DD as it has a wide range of clinical presentations, therefore going undiagnosed for many years.

PDF Share
  1. Darier's disease: Case report with oral manifestations. Med Oral Patol Oral Cir Bucal 2006;11:E404-06.
  2. Cell adhesion in Haily-Haily disease and Darier's disease immunocytological and explant-tissue-culture studies. Br J Dermatol 1991;125:426-58.
  3. The incidence and distribution of oral lesions in patients with Darier's disease. Br Dent J 1991;171: 133-36.
  4. Darier's disease in neurocutaneous disorders phakomatoses and hamartoneoplastic syndromes. Springer Verlag Gmbh (1st ed). Mexico 2008;649-59, DOI: 10.1007/978-3-211-69500-5_43.
  5. Induction of Darier-White disease with UVB radiation in a clinically photo-insensitive patient. J Am Acad Dermatol. May 1996;34(5 Pt 2):931-34 [Medline].
  6. Darier's disease affecting the gingival and oral mucosal surfaces. Oct 2006;102(4)OOOOE.
  7. Unilateral Darier's disease with unilateral guttate leukoderma. J Am Acad Dermatol 2003;48:955-57.
  8. Darier's disease: Variable clinical presentation and a fatal outcome. Clin Exp Dermatol Jul 2009;34(5):628-29 [Medline].
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.