IADVL
Indexed with PubMed and Science Citation Index (E) 
 
Users online: 3758 
     Home | Feedback | Login 
About Current Issue Archive Ahead of print Search Instructions Online Submission Subscribe What's New Contact  
  NAVIGATE Here 
    Next article
    Previous article
    Table of Contents

 RESOURCE Links
    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
    Viewed9265    
    Printed97    
    Emailed1    
    PDF Downloaded322    
    Comments [Add]    
    Cited by others 11    

Recommend this journal

 

 CASE REPORT
Year : 2007  |  Volume : 73  |  Issue : 1  |  Page : 36--39

Dorfman-Chanarin syndrome


1 Department of Dermatology and STD, University College of Medical Sciences and Guru Teg Bahadur Hospital, Delhi, India
2 Department of Pathology, University College of Medical Sciences and Guru Teg Bahadur Hospital, Delhi, India

Correspondence Address:
Vijay Gandhi
A-242, Surya Nagar, Ghaziabad - 201 011, UP
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0378-6323.30650

Rights and Permissions

A four-year-old girl was brought to the dermatology outpatient department with scaling all over the body since birth. She had history of episodic vomiting and abdominal distension. A dermatological diagnosis of lamellar ichthyosis was made. Abdominal examination revealed a nontender hepatomegaly, fatty liver on ultrasonography and deranged liver function tests. Peripheral blood smear showed lipid vacuoles in the granulocytes consistent with Jordans' anomaly. Similar lipid vacuoles were seen in the basal layer in skin biopsy. An inflammatory infiltrate, moderate fibrosis in the portal tract and diffuse severe fatty change in hepatocytes were seen in liver biopsy. The patient was diagnosed as a case of Dorfman-Chanarin syndrome.






[FULL TEXT] [PDF]*


        
Print this article     Email this article

Online since 15th March '04
Published by Wolters Kluwer - Medknow