Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contact Us Login 
An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists


 
CASE REPORT Table of Contents   
Year : 2017  |  Volume : 21  |  Issue : 2  |  Page : 321
Darier disease: A rare genodermatosis


1 Department of Oral Pathology and Microbiology, Chhattisgarh Dental College and Research Institute, Rajnandgaon, Chhattisgarh, India
2 Department of Oral Pathology and Microbiology, Rajasthan Dental College and Hospital, Jaipur, Rajasthan, India

Correspondence Address:
Pramod Kumar
House No. 1, Shakti Nagar, Mal Godam Road, Etawah - 206 001, Uttar Pradesh
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jomfp.JOMFP_170_16

Rights and Permissions

Darier disease (DD), also known as keratosis follicularis or dyskeratosis follicularis, is a rare autosomal dominant genodermatosis with high penetrance and variable expressivity. It is caused by mutations of ATP2A2 gene which encodes the sarco/endoplasmic reticulum Ca2+ ATPase isoform 2. It is clinically manifested by hyperkeratotic papules primarily affecting seborrheic areas on the head, neck and thorax, with less frequent involvement of the oral mucosa. When oral manifestations are present, they primarily affect the palatal and alveolar mucosa, are usually asymptomatic and are discovered in routine dental examination. Histologically, the lesions show suprabasal clefts with acantholytic and dyskeratotic cells. We present a case of 35-year-old female patient with typical clinical and histological features of DD.


[FULL TEXT] [PDF]*
Print this article  Email this article
    

  Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
  Related articles
   Citation Manager
  Access Statistics
   Reader Comments
   Email Alert *
   Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed5519    
    Printed48    
    Emailed0    
    PDF Downloaded254    
    Comments [Add]    

Recommend this journal

 

Journal of Oral and Maxillofacial Pathology | Published by Wolters Kluwer - Medknow
Online since 15th Aug, 2007