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      Cutis verticis gyrata secondary to cerebriform intradermal naevus: a rare entity and its management

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          Abstract

          Abstract Cutis verticis gyrata (CVG) is a rare skin disorder characterised by thick skin folds and grooves, resembling the gyri and sulci of the cerebral cortex. It can be classified as primary essential, primary non-essential and secondary CVG. The rarity of the condition poses a challenge in the diagnosis and ultimately requires good communication between the clinician and the dermatopathologist for diagnosis. Many names have been given to the condition, including Robert-Unna syndrome, cutis verticis plicata, paquidemia verticis gyrata, cutis sulcata, cutis striata or “bulldog” scalp syndrome. To date, the treatment is still limited to plastic surgeons with excision of the folds, scalp reduction and reconstruction. In this article, we are sharing a rare case and the surgical management of a middle age gentleman with cutis verticis gyrata secondary to cerebral intradermal nevus (CIN) over his occipital scalp.

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          Cutis verticis gyrata: three cases with different aetiologies that demonstrate the classification system.

          Cutis verticis gyrata describes a scalp condition where there are convoluted folds and deep furrows that resemble the surface of the cerebral cortex. We report three cases of cutis verticis gyrata that demonstrate the current classification structure. The first case is the most commonly described primary non-essential acquired form, appearing in the scalp of an 11-year-old girl with mental retardation. The second case is the primary non-essential congenital form, presenting at birth in a baby with Noonan's syndrome. An association between cutis verticis gyrata and Noonan's syndrome has been rarely described. The third case illustrates secondary cutis verticis gyrata, occurring in a 27-year-old man with discoid eczema.
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            Cerebriform intradermal nevus. A cause of cutis verticis gyrata.

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              Is cutis verticis Gyrata-Intellectual Disability syndrome an underdiagnosed condition? A case report and review of 62 cases.

              Cutis Verticis Gyrata-Intellectual Disability (CVG-ID) syndrome is a rare neurocutaneous syndrome characterized by intellectual disability and scalp folds and furrows that are typically absent at birth and are first noticed after puberty. First reported in 1893, the syndrome was mainly identified in subjects living in psychiatric institutions, where it was found to have a prevalence of up to 11.4%. Most patients were reported in the literature during the first half of the 20th century. CVG-ID is now a less reported and possibly under-recognized syndrome. Here, we report a patient with CVG-ID that was diagnosed using the novel approach of magnetic resonance imaging and we conduct a systematic review of all patients reported in the last 60 years, discussing the core clinical features of this syndrome. © 2016 Wiley Periodicals, Inc.
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                Author and article information

                Journal
                ijm
                Iberoamerican Journal of Medicine
                Iberoam J Med
                Hospital San Pedro (Logroño, La Rioja, Spain )
                2695-5075
                2695-5075
                2020
                : 2
                : 4
                : 391-394
                Affiliations
                [4] Kubang Kerian orgnameUniversiti Sains Malaysia orgdiv1Hospital Universiti Sains Malaysia, Health Campus orgdiv2Reconstructive Sciences Unit Malaysia
                [6] Serdang orgnameUniversiti Putra Malaysia orgdiv1Faculty of Medicine and Health Sciences orgdiv2Department of Pathology Malasia
                [3] Kota Bharu orgnameHospital Raja Perempuan Zainab II orgdiv1Plastic and Reconstructive Surgery Unit Malasia
                [1] Kubang Kerian orgnameUniversiti Sains Malaysia orgdiv1Hospital Universiti Sains Malaysia, Health Campus orgdiv2Reconstructive Sciences Unit Malaysia
                [2] Kota Bharu orgnameHospital Raja Perempuan Zainab II orgdiv1Plastic and Reconstructive Surgery Unit Malasia
                [5] Kota Bharu orgnameHospital Raja Perempuan Zainab II orgdiv1Department of Pathology Malasia
                Article
                S2695-50752020000400023 S2695-5075(20)00200400023
                10.5281/zenodo.4011601
                3fb4bb20-3873-4d7c-9365-009a33126679

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                : 01 September 2020
                : 08 August 2020
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 10, Pages: 4
                Product

                SciELO Spain

                Categories
                Case Report

                Cerebriform intradermal nevus,Bulldog scalp syndrome,Cutis verticis gyrata

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