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Year : 2019  |  Volume : 10  |  Issue : 4  |  Page : 494-495  

A case of peeling skin syndrome


Department of Dermatology, Venereology and Leprosy, Bharati Vidyapeeth Medical College and Hospital, Sangli, Maharashtra, India

Date of Web Publication28-Jun-2019

Correspondence Address:
Rakhi Soman
Department of Dermatology, Venereology and Leprosy, PG Resident Girl's Hostel, Room Number 3, Bharati Vidyapeeth Medical College and Hospital, Sangli - 416 414, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_233_18

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How to cite this article:
Ashtekar RS, Soman R, Purandare CS. A case of peeling skin syndrome. Indian Dermatol Online J 2019;10:494-5

How to cite this URL:
Ashtekar RS, Soman R, Purandare CS. A case of peeling skin syndrome. Indian Dermatol Online J [serial online] 2019 [cited 2019 Jul 23];10:494-5. Available from: http://www.idoj.in/text.asp?2019/10/4/494/255534



A 12-year-old girl child, born of third degree consanguineous marriage, presented with recurrent, spontaneous, pruritic, painless episodes of peeling of skin over her body since 8 years of age. Cutaneous examination revealed multiple areas of exfoliation and erosions all over the body including the face [Figure 1], [Figure 2], [Figure 3]. Few hyperpigmented patches were observed at the sites of old healed lesions [Figure 4] with sparing of palms, soles, and mucosae [Figure 5]. Histopathological examination showed parakeratosis and characteristic “cleft” separating stratum corneum from granular layer [Figure 6] and [Figure 7]. She was diagnosed as Generalized Peeling Skin Syndrome (PSS), Type A, Non-inflammatory variant.[1]
Figure 1: Neck showing superficial peeling

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Figure 2: Upper limb showing exfoliation of skin

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Figure 3: Chest showing skin peeling

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Figure 4: Hyperpigmented patches of old healed lesions on trunk

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Figure 5: Sparing of palms

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Figure 6: Histopathological examination showing “subcorneal cleft” separating stratum corneum from stratum granulosum (H and E, ×10), respectively

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Figure 7: Histopathological examination showing “subcorneal cleft” separating stratum corneum from stratum granulosum (H and E, ×40), respectively

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PSS is a rare autosomal recessive cutaneous genodermatosis classified into two forms: Acral PSS and Generalized PSS. The generalized form is further classified into three types: Type A Non-inflammatory, Type B Inflammatory, and Type C. The PSS type A involves single missense mutation in CHST8 encoding a Golgi transmembrane N-acetylgalactosamine-4-O-sulphotransferase causing intracellular cleavge of corneocytes, thereby leading to asymptomatic peeling of skin as in our patient. The Type B PSS is clinically characterized by congenital icthyosis or erythematous migratory patches with a peeling border, which explains the clinical similarities of PSS type B with Netherton syndrome.[2]

In general, the differential diagnoses of PSS include congenital icthyosis, Netherton's syndrome, and severe dermatitis-multiple allergies-metabolic wasting syndrome.[3] Treatment includes symptomatic treatment with topical emollients as no effective treatment has been reported till date.[4]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Bowden PE. Peeling skin syndrome: Genetic defects in late terminal differentiation of the epidermis. J Invest Dermatol 2011;131:561-4.  Back to cited text no. 1
    
2.
Kharfi M, Khaled A, Ammar D, Ezzine N, El Fekih N, Fazaa B, et al. Generalized peeling skin syndrome: Case report and review of the literature. Dermatol Online J 2010;16:1.  Back to cited text no. 2
    
3.
Griffiths CE, Barker J, Bleiker T, Chalmers R, Creamer D. Rook's Textbook of Dermatology. 9th ed., West Sussex, UK: John Wiley & Sons, Ltd; 2016.  Back to cited text no. 3
    
4.
Mukhopadhyay AK. Peeling skin syndrome. Indian J Dermatol Venereol Leprol 2004;70:115-6.  Back to cited text no. 4
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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