IADVL
Indexed with PubMed and Science Citation Index (E) 
 
Users online: 4580 
     Home | Feedback | Login 
About Current Issue Archive Ahead of print Search Instructions Online Submission Subscribe What's New Contact  
  Navigate here 
  Search
 
   Next article
   Previous article 
   Table of Contents
  
 Resource links
   Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
  Related articles
   [PDF Not available] *
   Citation Manager
   Access Statistics
   Reader Comments
   Email Alert *
   Add to My List *
* Registration required (free)  

 
  In this article
   Abstract
   Introduction
   Case Report
   Comments
   References
   Article Figures

 Article Access Statistics
    Viewed3192    
    Printed44    
    Emailed0    
    PDF Downloaded0    
    Comments [Add]    

Recommend this journal

 


 
CASE REPORT
Year : 1994  |  Volume : 60  |  Issue : 6  |  Page : 343-344

Progressive symmetric erythrokeratoderma associated with symmetric syndactylism



Correspondence Address:
A K Jaiswal


Login to access the Email id

Source of Support: None, Conflict of Interest: None


Rights and PermissionsRights and Permissions

  Abstract 

A case of progressive symmetric erythrokeratoderma (PSEK) associated with symmetric syndactylism is being reported. The interesting feature being the first time description of any association with PSEK.


Keywords: Progressive symmetric erythrokeratoderma, Syndactylism


How to cite this article:
Jaiswal A K. Progressive symmetric erythrokeratoderma associated with symmetric syndactylism. Indian J Dermatol Venereol Leprol 1994;60:343-4

How to cite this URL:
Jaiswal A K. Progressive symmetric erythrokeratoderma associated with symmetric syndactylism. Indian J Dermatol Venereol Leprol [serial online] 1994 [cited 2020 Feb 17];60:343-4. Available from: http://www.ijdvl.com/text.asp?1994/60/6/343/4099



  Introduction Top


Progressive symmetric erythrokeratoderma (PSEK) is a rare inherited cornification disorder first described by Darier in 1911 [1] and is characterized by symmetric erythernatous hyperkeratotic plaques. Fewer than 30 cases have been reported [2],[3] and to our knowledge, no association of this condition with other disorders has been reported so far. This is the first description of PSEK associated with syndactylism.


  Case Report Top


A 5-year-old boy was evaluated recently in our unit because of asymptomatic cutaneous lesions of 4 months duration. On examination he showed erythematous, palmoplantar keratoderma that was sharply marginated over the wrists and ankles, with erythematous hyperkeratotic plaques distributed on the back of the hands and feet, elbows, knees, tendo Achillis, malleoli and buttocks. The lesions were well demarcated and exhibited remarkable symmetry [Figure - 1][Figure - 2]. In addition syndactyly of both feet involving the two outer toes [Figure - 1] and keratosis pilaris lesions over legs and lower back [Figure - 2] were also noted. The abdomen, thorax and face were unaffected. Rest of the physical examination was unremarkable.

There was no family history of similar skin eruptions and/or congenital defects, and the patient's parents were not related.

All of the laboratory examination were within normal limits. X-rays of the feet showed no synostosis. Histologic examination of skin biopsy specimen from erythematous hyperkeratotic plaque revealed changes compatible with a diagnosis of PSEK.


  Comments Top


This case is doubly interesting not only in its rarity but also in its first time association with other disorders. Furthermore the associated syndactylism also shows a striking degree of symmetry akin to PSEK lesions. Finally, the triple association of PSEK, symmetric syndactylism and keratosis pilaris in the present case is just coincidental or actually features of a new syndrome?

 
  References Top

1.Darier J. Erythrokeratodermie Verruqueuse en nappes, symetrique et progressive. Bull Soc Fr Dermatol Syphiligr 1911; 22: 252-64.  Back to cited text no. 1    
2.Nazzaro V, Bla.ichet-Bardon C. Progressive symmetric erythrokeratodermia. Arch Dermatol 1986; 122: 434-40.  Back to cited text no. 2    
3.Gharpuray MB, Patki AH, Pandit P, Kulkarni V. Progressive symmetric erythrokerato­derma. Ind J Dermatol Venereol Leprol 1990; 56: 240-2.  Back to cited text no. 3    


    Figures

[Figure - 1], [Figure - 2]



 

Top
Print this article  Email this article
Previous article Next article

    

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