|THROUGH THE LENS
|Year : 2019 | Volume
| Issue : 4 | Page : 494-495
A case of peeling skin syndrome
Renuka Satish Ashtekar, Rakhi Soman, Chandrashekhar Shrihari Purandare
Department of Dermatology, Venereology and Leprosy, Bharati Vidyapeeth Medical College and Hospital, Sangli, Maharashtra, India
|Date of Web Publication||28-Jun-2019|
Department of Dermatology, Venereology and Leprosy, PG Resident Girl's Hostel, Room Number 3, Bharati Vidyapeeth Medical College and Hospital, Sangli - 416 414, Maharashtra
Source of Support: None, Conflict of Interest: None
|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
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.
|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.
In general, the differential diagnoses of PSS include congenital icthyosis, Netherton's syndrome, and severe dermatitis-multiple allergies-metabolic wasting syndrome. Treatment includes symptomatic treatment with topical emollients as no effective treatment has been reported till date.
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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]