• Users Online: 181
  • Print this page
  • Email this page
CASE REPORT
Year : 2020  |  Volume : 9  |  Issue : 2  |  Page : 120-123

An unusual case of dominant dystrophic epidermolysis bullosa (Cockayne-Touraine disease) associated with juvenile idiopathic arthritis


1 Department of Medicine, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
2 National Health Private Limited, JP Nagar, Bengaluru, Karnataka, India
3 Department of Pathology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India

Correspondence Address:
K M Bhargav
Assistant Professor, Department of Medicine, Sri Venkateswara Institute of Medical Sciences, Tirupati 517 507, Andhra Pradesh
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JCSR.JCSR_14_20

Rights and Permissions

We report a rare case of co-existence of dominant dystrophic epidermolysis bullosa (EB) and juvenile idiopathic arthritis (JIA) in an 18-year-old male patient, who is born out of consanguineous marriage. He presented with blistering skin lesions all over the body since the 2nd day after his birth, anonychia of all the toes from childhood and symmetric polyarticular inflammatory arthritis for 4 years. Erythrocyte sedimentation rate and C-reactive protein were elevated. Skin biopsy of induced vesicle was done, and histopathology showed epidermis with basket-weave hyperkeratosis, basal pigmented layer and sub-epidermal free bulla, while dermis revealed a few thin-walled proliferating capillaries with sparse lymphocytes surrounding them. Immunofluorescence showed immunoglobulin G along the basement membrane confirming the diagnosis of EB. He was diagnosed to have polyarticular rheumatoid factor-negative JIA. Treatment with oral methylprednisolone, disease-modifying anti-rheumatic drugs and intra-articular triamcinolone injections resulted in symptomatic relief of the joint pains.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 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
    Viewed74    
    Printed3    
    Emailed0    
    PDF Downloaded17    
    Comments [Add]    

Recommend this journal