Indexed with PubMed and Science Citation Index (E) 
Users online: 1647 
     Home | Feedback | Login 
About Current Issue Archive Ahead of print Search Instructions Online Submission Subscribe What's New Contact  
    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
    PDF Downloaded138    
    Comments [Add]    

Recommend this journal


Year : 2012  |  Volume : 78  |  Issue : 5  |  Page : 625--631

Peeling skin syndrome in eight cases of four different families from India and Bangladesh

1 Department of Dermatology, NRS Medical College, 138 AJC Bose Road, Kolkata, West Bengal, India
2 Department of Pathology, NRS Medical College, 138 AJC Bose Road, Kolkata, West Bengal, India

Correspondence Address:
Nilendu Sarma
P.N. Colony, Sapuipara, Bally, Howrah - 711 227, WB
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0378-6323.100583

Rights and Permissions

Peeling skin syndrome (PSS) is a rare recessively inherited ichthyosiform genodermatoses characterized by superficial skin peeling. This has 2 subtypes, acral (APSS; OMIM 609796) and generalized form (OMIM 270300). The later has been subdivided into type A (non-inflammatory) and type B (inflammatory). Eight cases of peeling skin syndrome in 4 families were recorded over a period of 5 years. They were diagnosed clinically and confirmed histopathologically. Disease onset ranged from birth to childhood age (mean 5.25 ± 4.528 years) and age at presentation ranged from 7-35 years (mean 23.25 ± 10.471 years). Males outnumbered females (M:F - 5:3). All had non-inflammatory generalized disease of type-A PSS variety, except one who had type-B PSS. Two Muslim families (1 st and 2 nd family, total 5 patients) came from nearby country Bangladesh, and the 2 Hindu families were Indian. Higher severity over acral areas in generalized type, possible autosomal dominant pattern of inheritance and improvement with age as found in this series were new manifestations and possibly unreported previously. The disease was found to be poorly responsive to oral retinoids. Prevalence of the disease may be higher than expected. Importance of mutational analysis was also highlighted.


Print this article     Email this article

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