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Suh, Á. Flórez-Menéndez, C. de La Torre-Fraga" "autores" => array:3 [ 0 => array:4 [ "nombre" => "H." "apellidos" => "Suh" "email" => array:2 [ 0 => "suhhaejin@gmail.com" 1 => "hsuhoh@yahoo.es" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "Á." "apellidos" => "Flórez-Menéndez" ] 2 => array:2 [ "nombre" => "C." "apellidos" => "de La Torre-Fraga" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Dermatología, Complejo Hospitalario Universitario de Pontevedra, Pontevedra, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Forma familiar de <span class="elsevierStyleItalic">cutis verticis gyrata</span> primario esencial" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 932 "Ancho" => 1800 "Tamanyo" => 288970 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Anterior-posterior folding of the scalp (patient<span class="elsevierStyleHsp" style=""></span>1). B, Anterior-posterior folding of the scalp (patient<span class="elsevierStyleHsp" style=""></span>2).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Cutis verticis gyrata (CVG) is a rare skin condition characterized by thickening of the scalp, giving rise to convolutions and sulci, with a cerebriform pattern. CVG is classified as primary (essential and nonessential) or secondary.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We present 2 cases of primary essential CVG in a single family. The 2 men (patients 1 and 2) were siblings of 32 and 28 years of age, with no other relevant family history. They presented progressive thickening of the scalp that had started in adolescence. Physical examination revealed anterior-posterior folds on the scalp of both patients (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>) and mild subungual hyperkeratosis and distal onycholysis of the nail plates of the great toes of both feet.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Detailed dermatologic and systemic medical history and physical examination, including endocrine and neurologic evaluation, detected none of the abnormalities associated with the primary nonessential forms. Scalp biopsy revealed a mild nonspecific superficial periadnexal and perivascular mononuclear inflammatory infiltrate in both patients. The following studies were performed to exclude causes of secondary CVG: plain x-rays of both hands, cerebral computed tomography (CT), routine blood tests with thyroid function, lipid profile, insulin-like growth factor<span class="elsevierStyleHsp" style=""></span>1, antinuclear antibodies, and syphilis serology. The cerebral CT of patient 1 showed the skin folds affecting the scalp (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Other studies were normal or negative. Studies of the nail alterations (culture and histopathology) were only positive in patient 2 (growth of <span class="elsevierStyleItalic">Trichophyton interdigitale</span>), and treatment was prescribed with oral terbinafine (250<span class="elsevierStyleHsp" style=""></span>mg/24<span class="elsevierStyleHsp" style=""></span>h for 3 months). Genetic analysis was not performed in either patient.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Primary essential CVG is most common in postpubertal men.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">1</span></a> The etiology is unknown, but genetic and endocrine factors are believed to be implicated.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">2</span></a> The majority of cases of primary essential CVG are sporadic, although familial forms with autosomal dominant or recessive inheritance with variable expression have been reported.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">3,4</span></a> The prevalence data for the general population date from 1964, with an estimated prevalence among men and women of 1 and 0.026 cases per 100<span class="elsevierStyleHsp" style=""></span>000 population, respectively.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">5</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">The primary essential form is not associated with other abnormalities, in contrast to the primary nonessential form, which can be associated with mental retardation, cerebral palsy, epilepsy, schizophrenia, deafness, and cranial and ophthalmologic abnormalities.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">6</span></a> The secondary forms of CVG can be associated with numerous underlying diseases, such as pachydermoperiostosis, acromegaly, cutis laxa, cylindroma, cerebriform intradermal nevus, amyloidosis, myxedema, syphilis, intracranial aneurysms, intraventricular ependymoma, and inflammatory conditions of the scalp; exclusion of these associations is therefore important (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>).<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">7,8</span></a></p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Primary CVG affects the scalp, particularly at the vertex and in the occipital region. The folds usually run symmetrically in an anterior-posterior direction, although they can be transverse in the occipital region. The number of folds varies between 2 and 12, and they cannot be corrected by pressure or traction.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">4</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The additional tests to be performed will depend on the clinical presentation and on any associated disease. Imaging studies such as magnetic resonance imaging or CT must be performed when neurologic or ophthalmologic abnormalities or mental retardation are present, although some authors recommend routinely performing these studies to exclude structural cerebral alterations.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">9</span></a> Scalp biopsy enables us to exclude other underlying causes, such as cerebriform intradermal nevus, cylindroma, and inflammatory dermatoses of the scalp. In the primary forms, histopathology is normal in the majority of cases, although thickening of the dermis and hypertrophy of the pilosebaceous units have been reported.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">10</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">CVG is a progressive disease and, in its primary essential form, only affects the scalp; surgery is therefore only considered in cases with major psychological repercussions.<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">11</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">In summary, we have presented a case of familial primary essential CVG. Primary essential CVG is a rare entity, especially in its familial form, with only 1 other familial case described, also with clinical manifestation arising in adolescence.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">4</span></a> In these cases it is still important to exclude causes of secondary CVG as well as diseases associated with the primary nonessential form.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of Interest</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of Interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Suh H, Flórez-Menéndez Á, de La Torre-Fraga C. Forma familiar de <span class="elsevierStyleItalic">cutis verticis gyrata</span> primario esencial. Actas Dermosifiliogr. 2016;107:435–437.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 932 "Ancho" => 1800 "Tamanyo" => 288970 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Anterior-posterior folding of the scalp (patient<span class="elsevierStyleHsp" style=""></span>1). B, Anterior-posterior folding of the scalp (patient<span class="elsevierStyleHsp" style=""></span>2).</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 968 "Ancho" => 950 "Tamanyo" => 67661 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Cerebral computed tomography showing irregular skin folds (patient<span class="elsevierStyleHsp" style=""></span>1).</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 2170 "Ancho" => 2753 "Tamanyo" => 280457 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Diagnostic algorithm.</p> <p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">CVG indicates cutis verticis gyrata.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:11 [ 0 => array:3 [ "identificador" => "bib0060" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Primary essential cutis verticis gyrata-case report" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "J.J. 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Year/Month | Html | Total | |
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2024 November | 23 | 8 | 31 |
2024 October | 150 | 41 | 191 |
2024 September | 169 | 26 | 195 |
2024 August | 208 | 56 | 264 |
2024 July | 190 | 28 | 218 |
2024 June | 183 | 25 | 208 |
2024 May | 180 | 38 | 218 |
2024 April | 156 | 23 | 179 |
2024 March | 191 | 26 | 217 |
2024 February | 167 | 28 | 195 |
2024 January | 207 | 32 | 239 |
2023 December | 226 | 21 | 247 |
2023 November | 238 | 45 | 283 |
2023 October | 247 | 28 | 275 |
2023 September | 260 | 39 | 299 |
2023 August | 157 | 26 | 183 |
2023 July | 179 | 51 | 230 |
2023 June | 123 | 27 | 150 |
2023 May | 161 | 27 | 188 |
2023 April | 145 | 25 | 170 |
2023 March | 155 | 34 | 189 |
2023 February | 143 | 36 | 179 |
2023 January | 130 | 29 | 159 |
2022 December | 144 | 47 | 191 |
2022 November | 103 | 24 | 127 |
2022 October | 77 | 30 | 107 |
2022 September | 90 | 41 | 131 |
2022 August | 111 | 32 | 143 |
2022 July | 127 | 40 | 167 |
2022 June | 117 | 30 | 147 |
2022 May | 211 | 31 | 242 |
2022 April | 202 | 24 | 226 |
2022 March | 163 | 36 | 199 |
2022 February | 187 | 39 | 226 |
2022 January | 245 | 56 | 301 |
2021 December | 130 | 42 | 172 |
2021 November | 166 | 48 | 214 |
2021 October | 112 | 37 | 149 |
2021 September | 101 | 39 | 140 |
2021 August | 149 | 36 | 185 |
2021 July | 92 | 38 | 130 |
2021 June | 96 | 25 | 121 |
2021 May | 108 | 36 | 144 |
2021 April | 157 | 52 | 209 |
2021 March | 73 | 30 | 103 |
2021 February | 63 | 21 | 84 |
2021 January | 41 | 8 | 49 |
2020 December | 28 | 14 | 42 |
2020 November | 33 | 19 | 52 |
2020 October | 49 | 16 | 65 |
2020 September | 19 | 4 | 23 |
2020 August | 38 | 13 | 51 |
2020 July | 30 | 11 | 41 |
2020 June | 39 | 24 | 63 |
2020 May | 19 | 14 | 33 |
2020 April | 33 | 17 | 50 |
2020 March | 22 | 14 | 36 |
2020 February | 6 | 0 | 6 |
2020 January | 2 | 0 | 2 |
2019 December | 5 | 0 | 5 |
2019 November | 2 | 0 | 2 |
2019 September | 6 | 0 | 6 |
2019 August | 2 | 0 | 2 |
2019 July | 2 | 0 | 2 |
2019 June | 3 | 0 | 3 |
2019 May | 3 | 2 | 5 |
2019 April | 1 | 4 | 5 |
2019 March | 2 | 4 | 6 |
2019 February | 3 | 0 | 3 |
2018 December | 8 | 0 | 8 |
2018 November | 8 | 0 | 8 |
2018 October | 4 | 0 | 4 |
2018 September | 9 | 0 | 9 |
2018 February | 43 | 1 | 44 |
2018 January | 63 | 6 | 69 |
2017 December | 68 | 6 | 74 |
2017 November | 46 | 6 | 52 |
2017 October | 54 | 6 | 60 |
2017 September | 39 | 7 | 46 |
2017 August | 30 | 5 | 35 |
2017 July | 17 | 11 | 28 |
2017 June | 33 | 7 | 40 |
2017 May | 21 | 13 | 34 |
2017 April | 23 | 7 | 30 |
2017 March | 15 | 27 | 42 |
2017 February | 11 | 10 | 21 |
2017 January | 18 | 7 | 25 |
2016 December | 30 | 18 | 48 |
2016 November | 23 | 12 | 35 |
2016 October | 28 | 15 | 43 |
2016 June | 0 | 2 | 2 |
2016 May | 0 | 1 | 1 |
2016 April | 0 | 4 | 4 |