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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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año/Mes | Html | Total | |
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2024 Noviembre | 11 | 2 | 13 |
2024 Octubre | 150 | 41 | 191 |
2024 Septiembre | 169 | 26 | 195 |
2024 Agosto | 208 | 56 | 264 |
2024 Julio | 190 | 28 | 218 |
2024 Junio | 183 | 25 | 208 |
2024 Mayo | 180 | 38 | 218 |
2024 Abril | 156 | 23 | 179 |
2024 Marzo | 191 | 26 | 217 |
2024 Febrero | 167 | 28 | 195 |
2024 Enero | 207 | 32 | 239 |
2023 Diciembre | 226 | 21 | 247 |
2023 Noviembre | 238 | 45 | 283 |
2023 Octubre | 247 | 28 | 275 |
2023 Septiembre | 260 | 39 | 299 |
2023 Agosto | 157 | 26 | 183 |
2023 Julio | 179 | 51 | 230 |
2023 Junio | 123 | 27 | 150 |
2023 Mayo | 161 | 27 | 188 |
2023 Abril | 145 | 25 | 170 |
2023 Marzo | 155 | 34 | 189 |
2023 Febrero | 143 | 36 | 179 |
2023 Enero | 130 | 29 | 159 |
2022 Diciembre | 144 | 47 | 191 |
2022 Noviembre | 103 | 24 | 127 |
2022 Octubre | 77 | 30 | 107 |
2022 Septiembre | 90 | 41 | 131 |
2022 Agosto | 111 | 32 | 143 |
2022 Julio | 127 | 40 | 167 |
2022 Junio | 117 | 30 | 147 |
2022 Mayo | 211 | 31 | 242 |
2022 Abril | 202 | 24 | 226 |
2022 Marzo | 163 | 36 | 199 |
2022 Febrero | 187 | 39 | 226 |
2022 Enero | 245 | 56 | 301 |
2021 Diciembre | 130 | 42 | 172 |
2021 Noviembre | 166 | 48 | 214 |
2021 Octubre | 112 | 37 | 149 |
2021 Septiembre | 101 | 39 | 140 |
2021 Agosto | 149 | 36 | 185 |
2021 Julio | 92 | 38 | 130 |
2021 Junio | 96 | 25 | 121 |
2021 Mayo | 108 | 36 | 144 |
2021 Abril | 157 | 52 | 209 |
2021 Marzo | 73 | 30 | 103 |
2021 Febrero | 63 | 21 | 84 |
2021 Enero | 41 | 8 | 49 |
2020 Diciembre | 28 | 14 | 42 |
2020 Noviembre | 33 | 19 | 52 |
2020 Octubre | 49 | 16 | 65 |
2020 Septiembre | 19 | 4 | 23 |
2020 Agosto | 38 | 13 | 51 |
2020 Julio | 30 | 11 | 41 |
2020 Junio | 39 | 24 | 63 |
2020 Mayo | 19 | 14 | 33 |
2020 Abril | 33 | 17 | 50 |
2020 Marzo | 22 | 14 | 36 |
2020 Febrero | 6 | 0 | 6 |
2020 Enero | 2 | 0 | 2 |
2019 Diciembre | 5 | 0 | 5 |
2019 Noviembre | 2 | 0 | 2 |
2019 Septiembre | 6 | 0 | 6 |
2019 Agosto | 2 | 0 | 2 |
2019 Julio | 2 | 0 | 2 |
2019 Junio | 3 | 0 | 3 |
2019 Mayo | 3 | 2 | 5 |
2019 Abril | 1 | 4 | 5 |
2019 Marzo | 2 | 4 | 6 |
2019 Febrero | 3 | 0 | 3 |
2018 Diciembre | 8 | 0 | 8 |
2018 Noviembre | 8 | 0 | 8 |
2018 Octubre | 4 | 0 | 4 |
2018 Septiembre | 9 | 0 | 9 |
2018 Febrero | 43 | 1 | 44 |
2018 Enero | 63 | 6 | 69 |
2017 Diciembre | 68 | 6 | 74 |
2017 Noviembre | 46 | 6 | 52 |
2017 Octubre | 54 | 6 | 60 |
2017 Septiembre | 39 | 7 | 46 |
2017 Agosto | 30 | 5 | 35 |
2017 Julio | 17 | 11 | 28 |
2017 Junio | 33 | 7 | 40 |
2017 Mayo | 21 | 13 | 34 |
2017 Abril | 23 | 7 | 30 |
2017 Marzo | 15 | 27 | 42 |
2017 Febrero | 11 | 10 | 21 |
2017 Enero | 18 | 7 | 25 |
2016 Diciembre | 30 | 18 | 48 |
2016 Noviembre | 23 | 12 | 35 |
2016 Octubre | 28 | 15 | 43 |
2016 Junio | 0 | 2 | 2 |
2016 Mayo | 0 | 1 | 1 |
2016 Abril | 0 | 4 | 4 |