Indexed with PubMed and Science Citation Index (E) 
Users online: 2352 
     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
   Article in PDF (97 KB)
   Citation Manager
   Access Statistics
   Reader Comments
   Email Alert *
   Add to My List *
* Registration required (free)  

  In this article

 Article Access Statistics
    PDF Downloaded288    
    Comments [Add]    
    Cited by others 2    

Recommend this journal

Year : 2004  |  Volume : 70  |  Issue : 2  |  Page : 115-116

Peeling skin syndrome

"Pranab", Ismile (Near Dharmaraj Mandir), P.O. Asansol - 713301

Correspondence Address:
"Pranab", Ismile (Near Dharmaraj Mandir), P.O. Asansol - 713301
[email protected]

How to cite this article:
Mukhopadhyay AK. Peeling skin syndrome. Indian J Dermatol Venereol Leprol 2004;70:115-6

How to cite this URL:
Mukhopadhyay AK. Peeling skin syndrome. Indian J Dermatol Venereol Leprol [serial online] 2004 [cited 2021 Jan 20];70:115-6. Available from:

Peeling skin syndrome (PSS) is a rare disorder characterized by continual shedding of the entire stratum corneum. It starts either at birth or later in childhood.[1] It is inherited in an autosomal recessive pattern.[2] The skin involvement is usually generalized, but in some patients sparing of the face, palms and soles has been reported.[3] Here we report a case of PSS in a girl who had sparing of palms and soles and whose disease showed summer exacerbation.

A 5-year-old girl presented with a history of peeling of skin from all over the body in patches since 1 year of age. She was born of a non-consanguineous marriage and there was no history of a similar disorder in any of her family members. Her problem of peeling of the skin remained all around the year but worsened in the summer. The lesions were mildly itchy. On examination she had patches of peeling skin all over the body along with a few lesions on her face [Figure - 1]. Rubbing the normal skin revealed the peeling but there was no oozing from the rubbed area. Some hyperpigmented patches remained at the sites of old lesions. The palms, soles and mucous membranes were uninvolved. General and systemic examination revealed no abnormality. Routine hemogram and urinalysis were normal. Histopathological examination of the skin showed separation of the stratum corneum away from the stratum granulosum, mild acanthosis and a normal dermis [Figure - 2]. The patient was treated with emollients which gave her symptomatic relief to some extent, but on follow up she had recurrence of the lesions.

PSS is variously known as keratolysis exfoliativa, congenital deciduous skin, and familial continual skin peeling.[4] Troupe has pointed out that there are two types of peeling skin syndrome. The first was first described by Fox in 1921, and is characterized by shedding of the entire skin including the face, comparable to the shedding of skin by reptiles. The peeling occurs in the lower part of the stratum corneum. The second type, first described by Wile in 1924, presents with erythroderma at birth, and is associated with features such as growth retardation, aminoaciduria, etc. The epidermis is psoriasiform with an absent or reduced granular layer and with marked parakeratosis. The split occurs at the level of the granular layer.[2] It has been suggested that the basic lesion is located at the stratum corneum-granular layer junction; ultrastructually, intercellular disruption occurs above the lower two layers of the stratum corneum within the stratum lucidum.[4]

The cause of this disorder is unknown.[5] It has been postulated that the defect is reduced adherence of abnormally thick stratum corneum to the stratum granulosum.[6] In some patients, association of easy pluckability of hair, shedding of nails, hypogonadism and anosmia has been described.[4] Usually there are no seasonal exacerbations, but some patients appear to worsen in summer.[5] As far as treatment is concerned, emollients such as petrolatum jelly and salicylic acid gel may be helpful in improving the appearance.[7] Drugs derived from vitamin A, such as tretinoin and etretinate, could be effective.[8] In the present case, the history of exacerbation during the summer and sparing of the palms and soles are uncommon and interesting features. 

   References Top

1.Sparker MK. Differential diagnosis of neonatal erythroderma. In: Harper J, Oranje A, Prose N, editors. Textbook of pediatric dermatology. Oxford: Blackwell Science; 2000. p. 92-103.  Back to cited text no. 1    
2.Braun-Falco O, Plewig G, Wolff HH, Burgdorf WHC. Dermatology. 2nd ed. Berlin: Springer-Verlag; 2000.  Back to cited text no. 2    
3.Sharma R, Kumar A. Skin peeling syndrome. Indian J Dermatol Venereol Leprol 2000;66:154-5.  Back to cited text no. 3    
4.Griffiths WAD, Judge MR, Leigh IM. Disorders of keratinisation. In: Champion RH, Burton JL, Burns DA, Breathnach SM, editors. Textbook of dermatology. 6th ed. Oxford: Blackwell Science; 1998. p. 1530-2.  Back to cited text no. 4    
5.Dicken CH. Peeling skin syndrome. J Am Acad Dermatol 1985;13:158-60.  Back to cited text no. 5  [PUBMED]  
6.Mevorah B, Frenk E, Saurat JH, Siegenthaler G. Peeling skin syndrome: A clinical, ultrastructural and biochemical study. Br J Dermatol 1987;116:117-25.  Back to cited text no. 6  [PUBMED]  
7.Panja SK, Sengupta S. Idiopathic deciduous skin. Int J Dermatol 1982;21:262-4.  Back to cited text no. 7  [PUBMED]  
8.National Organization for Rare Disorders. (Report). Peeling skin syndrome., updated 26/01/1998, accessed.  Back to cited text no. 8    


Print this article  Email this article
Previous article Next article


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