Biophysical profile of a case of Peeling Skin Syndrome – A model for study of Stratum Corneum function
Abstract
Peeling skin syndrome (PSS) is a rare recessively inherited ichthyosiform genodermatoses characterized clinically by asymptomatic, localized or generalized, continuous exfoliation of the superficial layer of the skin. A 12-year-old male child was presented with history of asymptomatic peeling of skin from his neck, trunk, and proximal extremities, since the age of 6 years. Histologically, stratum corneum was separated from the underlying stratum granulosum. Clinical and histopathological picture corresponded to the non-inflammatory variant of peeling skin syndrome (type A). Biophysical profile, which includes skin hydration, transepidermal water loss, sebum content, skin pH, of this patient was assessed from 7 different sites using the Corneometer, Tewameter, Sebumeter, Skin-pH-meter. Derangement was observed in all the above parameters. Peeling skin syndrome serves as a model for studying the stratum corneum functions. This article gives a critical review of the structure and various barrier functions of stratum corneum.
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Introduction
Peeling skin syndrome (PSS) is a rare recessively inherited ichthyosiform genodermatoses characterized clinically by asymptomatic, localized or generalized, continuous exfoliation of the superficial layer of the stratum corneum (SC). It has 2 subtypes, acral and generalized form. The later has been subdivided into type A (non-inflammatory)1 and type B (inflammatory) 2 Mevorah et al3 introduced a new subtype, type C3 , which starts in infancy and characterized by atopy, itching, and presence of circular erythematous patches that are encircled by areas of peeling. Histologically it is characterized by separation in the intracorneal or subcorneal layers of the epidermis. 4 Etiology of this disorder is unknown. 5 It has been postulated that this is due to reduced adherence of abnormally thick stratum corneum to the stratum granulosum. 3 Total number of cases of PSS reported so far is 81 and it consisted of 20 cases of type A PSS, 27 cases of type B PSS, 2 cases of type C PSS, 21 cases of acral PSS, and 11 of undefined phenotypes. 1,3
Biophysical profile is a bioengineering method to objectively assess the skin function. Stratum corneum function assessment is done by measuring 4 parameters : Skin hydration, measured by Corneometer CM 825; Skin sebum content, measured by Sebumeter SM 815; Skin barrier function or transepidermal water loss (TEWL), measured by Tewameter Triple TM 330T; Skin pH, measured by Skin-pH-Meter PH 905 (supplied by the manufacturer, Courage & Khazaka electronic GmbH, Cologne, Germany).
Peeling skin syndrome (PSS) serves as a model for studying the stratum corneum functions, as it involves separation of stratum corneum.
Conclusion
Peeling skin syndrome (PSS) serves as a model for studying the stratum corneum functions, as it involves separation of stratum corneum.