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      Nevus Comedonicus Syndrome

      case-report

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          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          A case of nevus comedonicus syndrome with atypical cutaneous presentation (widespread involvement without any particular pattern, midline lesions involving lower abdomen and involvement of bilateral pinna), and some unusual skeletal (adduction deformity involving bilateral metatarsal along with medial deviation at the level of tarsometatarsal joint), central nervous system (agenesis of corpus callosum with a interhemispheric cyst), visceral (pancreatic cyst) and neurological manifestations have been illustrated.

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          The group of epidermal nevus syndromes Part I. Well defined phenotypes.

          Rudolf Happle (2010)
          The epidermal nevus syndromes represent a group of distinct disorders that can be distinguished by the type of associated epidermal nevus and by the criterion of presence or absence of heritability. Well defined syndromes characterized by organoid epidermal nevi include Schimmelpenning syndrome, phacomatosis pigmentokeratotica, nevus comedonicus syndrome, angora hair nevus syndrome, and Becker nevus syndrome. The molecular basis of these disorders has so far not been identified. By contrast, the group of syndromes characterized by keratinocytic nevi comprises three phenotypes with a known molecular etiology in the form of CHILD (congenital hemidysplasia with ichthyosiform nevus and limb defects) syndrome, type 2 segmental Cowden disease, and fibroblast growth factor receptor 3 epidermal nevus syndrome (García-Hafner-Happle syndrome), whereas Proteus syndrome is still of unknown origin. From this overview, it is clear that a specific type of these disorders cannot be classified by the name "epidermal nevus syndrome" nor by the terms "organoid nevus syndrome" or "keratinocytic nevus syndrome." After completing this learning activity, participants should be able to distinguish nine different epidermal nevus syndromes by their characteristic features, understand the practical significance of avoiding terms like "epidermal nevus syndrome" or "keratinocytic nevus syndrome" to define any specific entity within this group of disorders, and differentiate between nonhereditary traits and those bearing a genetic risk because of either Mendelian or non-Mendelian inheritance. Copyright (c) 2010 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.
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            Naevus comedonicus: a spectrum of body involvement.

            A Deng, B Sina, Kjetil K S Guldbakke … (2007)
            Naevus comedonicus (NC) is a rare developmental anomaly, with < 200 cases reported in the literature. It usually occurs on the face, neck or chest, appearing as groups of closely arranged dilated follicular openings with keratin plugs. Several associations have been made in the literature. We review the current literature, emphasizing the clinical features, associated conditions and therapeutic options.
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              Nevus comedonicus syndrome: a case associated with multiple basal cell carcinomas and a rudimentary toe.

              Ertan Yilmaz, Cicek Durusoy, G Karpuzoglu … (2005)
              Article 0 comments Cited 6 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Indian J Dermatol
                Journal ID (iso-abbrev): 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): Jul-Aug 2015
                Volume: 60
                Issue: 4
                Page: 421
                Affiliations
                [1] From the Department of Dermatology and Sexually Transmitted Diseases, Lady Hardinge Medical College and Suchita Kriplani Hospital, Shaheed Bhagat Singh Marg, Delhi - 110 001, India
                Author notes
                Address for correspondence: Dr. Pravesh Yadav, RZ-97, Phase-III, Prem Nagar, Najafgarh, New Delhi - 110 043, India. E-mail: rao.pravesh@ 123456gmail.com
                Article
                Publisher ID: IJD-60-421c
                DOI: 10.4103/0019-5154.160523
                PMC ID: 4533567
                SO-VID: 80c57fb9-16b1-4b61-80c5-7f28a5f5cea0
                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 : March 2014
                Date accepted : November 2014
                Categories
                Subject: E-IJD Case Report

                ScienceOpen disciplines: Dermatology
                Keywords: comedo nevus,nevus comedonicus syndrome,nevus comedonicus
                Data availability:
                ScienceOpen disciplines: Dermatology
                Keywords: comedo nevus, nevus comedonicus syndrome, nevus comedonicus

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