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      KINDLER'S SYNDROME: A CASE SERIES OF THREE INDIAN CHILDREN

      case-report

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          Abstract

          Kindler's syndrome is a very rare genodermatosis characterized by acral blistering starting in infancy, photosensitivity, progressive poikiloderma, cutaneous atrophy, and various forms of mucosal involvement. A large number of other cutaneous and extracutaneous features have also been described. We report here three cases of Kindler's syndrome from eastern India for the rarity of the syndrome and to emphasize the importance of considering this condition in the differential diagnosis of disorders that can cause blistering, cutaneous atrophy, and/or poikilodermatous skin changes.

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          Loss of kindlin-1, a human homolog of the Caenorhabditis elegans actin-extracellular-matrix linker protein UNC-112, causes Kindler syndrome.

          Fenella Wojnarowska, Julia Wiebe, Ilona Frieden … (2003)
          Kindler syndrome is an autosomal recessive disorder characterized by neonatal blistering, sun sensitivity, atrophy, abnormal pigmentation, and fragility of the skin. Linkage and homozygosity analysis in an isolated Panamanian cohort and in additional inbred families mapped the gene to 20p12.3. Loss-of-function mutations were identified in the FLJ20116 gene (renamed "KIND1" [encoding kindlin-1]). Kindlin-1 is a human homolog of the Caenorhabditis elegans protein UNC-112, a membrane-associated structural/signaling protein that has been implicated in linking the actin cytoskeleton to the extracellular matrix (ECM). Thus, Kindler syndrome is, to our knowledge, the first skin fragility disorder caused by a defect in actin-ECM linkage, rather than keratin-ECM linkage.
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            Congenital poikiloderma with traumatic bulla formation and progressive cutaneous atrophy.

            T KINDLER (1954)
            Article 0 comments Cited 37 times – based on 0 reviews     Review now
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              Kindler syndrome: a case report and proposal for clinical diagnostic criteria.

              Irena Angelova Fischer, Jana Kazandjieva, Snejina Vassileva … (2005)
              Kindler syndrome is a rare hereditary disorder characterized by acral blister formation in infancy and childhood, progressive poikiloderma, cutaneous atrophy and increased photosensitivity. Since it was first described in 1954, less than 100 cases have been reported worldwide. Recently it has been reported that Kindler syndrome is the first genodermatosis caused by a defect in the actin-extracellular matrix linkage, and the gene was mapped to chromosome 20p12.3. The clinical features of the syndrome have been annotated by different authors but the definite of criteria to confirm the diagnosis have not yet been generally accepted. We report a case of Kindler syndrome that presents a full spectrum of clinical manifestations, and we propose a set of clinical criteria for diagnosis.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Indian J Dermatol
                Journal ID (publisher-id): IJD
                Title: Indian Journal of Dermatology
                Publisher: Medknow Publications & Media Pvt Ltd (India )
                ISSN (Print): 0019-5154
                ISSN (Electronic): 1998-3611
                Publication date (Print): Oct-Dec 2010
                Volume: 55
                Issue: 4
                Pages: 393-396
                Affiliations
                [1] From the Department of Dermatology, Venereology, and Leprosy, R.G.Kar Medical College, Kolkata, India
                [1 ] Department of Dermatology, School of Tropical Medicine, Kolkata, India
                Author notes
                Address for correspondence: Dr. Sudip Kumar Ghosh, Vill. and P.O-Rajballavpur (via-Maslandpur), District - 24 Parganas (North) - 743289, West Bengal, India dr_skghosh@ 123456yahoo.co.in
                Article
                Publisher ID: IJD-55-393
                DOI: 10.4103/0019-5154.74568
                PMC ID: 3051307
                PubMed ID: 21430900
                SO-VID: 4e72c7c2-b0c4-4ae1-9414-0291cbfada10
                Copyright © Copyright: © Indian Journal of Dermatology
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : October 2008
                Date accepted : March 2009
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Dermatology
                Keywords: poikiloderma,photosensitivity,kindlers’ syndrome
                Data availability:
                ScienceOpen disciplines: Dermatology
                Keywords: poikiloderma, photosensitivity, kindlers’ syndrome

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