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Peeling skin diseases: 21 cases from Turkey and a review of the literature.
MedLine Citation:
PMID:  21711468     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Background  Peeling skin diseases (PSD) refer to a group of rare autosomal recessive dermatosis which are characterized by spontaneous, continual peeling of the skin. Three different clinical pictures can be distinguished: Inflammatory PSD also referred to as peeling skin syndrome (PSS) type B, non-inflammatory PSD also referred to as PSS type A, and localized forms i.e. acral type PSS. Objective  To characterize the clinical and histopathological features of PSD in Turkey. Methods  We retrospectively reviewed the medical records and clinical photographs of patients who were given diagnosis of PSD and conducted histopathological evaluation of skin biopsies to identify the site of cleavage. Also we evaluated the cases including age, gender, age onset, clinical and histological findings, family history, associated disorders and PSD type. Results  Twenty-one patients with PSD were seen at Gulhane School of Medicine in Ankara between the years 1994 and 2010 in this retrospective study. All patients were men. Their ages were between 20 and 26 years (22.44 ± 2.30, Mean age ± SD). Of the patients, eight cases (40%) were type A, eight cases (40%) were type B, and five cases (20%) were acral type PSS. Eleven cases (52%) had parental consanguinity. Keratoderma, cheilitis, keratosis pilaris, melanonichia, clubbing, hyperhidrosis, onychodystrophy were observed in eight cases as an accompanying disorder. Conclusions  In this case series, PSD occurred rarely and also showed generally mild course of disease in Turkey and most likely related to consanguineous of marriages. Future investigations on PSD will contribute to our progressing alternative targets for pathogenesis-based therapy.
Authors:
O Köse; M Safali; E Koç; E Arca; G Açikgöz; I Ozmen; Y Yeniay
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-6-29
Journal Detail:
Title:  Journal of the European Academy of Dermatology and Venereology : JEADV     Volume:  -     ISSN:  1468-3083     ISO Abbreviation:  -     Publication Date:  2011 Jun 
Date Detail:
Created Date:  2011-6-29     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9216037     Medline TA:  J Eur Acad Dermatol Venereol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2011 The Authors. Journal of the European Academy of Dermatology and Venereology © 2011 European Academy of Dermatology and Venereology.
Affiliation:
Department of Dermatology, Gulhane Military Medical Academy, School of Medicine Pathology, Ankara, Turkey.
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