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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Cutis verticis gyrata &#40;CVG&#41; is a rare skin condition characterized by thickening of the scalp&#44; giving rise to convolutions and sulci&#44; with a cerebriform pattern&#46; CVG is classified as primary &#40;essential and nonessential&#41; or secondary&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">We present 2 cases of primary essential CVG in a single family&#46; The 2 men &#40;patients 1 and 2&#41; were siblings of 32 and 28 years of age&#44; with no other relevant family history&#46; They presented progressive thickening of the scalp that had started in adolescence&#46; Physical examination revealed anterior-posterior folds on the scalp of both patients &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; and mild subungual hyperkeratosis and distal onycholysis of the nail plates of the great toes of both feet&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Detailed dermatologic and systemic medical history and physical examination&#44; including endocrine and neurologic evaluation&#44; detected none of the abnormalities associated with the primary nonessential forms&#46; Scalp biopsy revealed a mild nonspecific superficial periadnexal and perivascular mononuclear inflammatory infiltrate in both patients&#46; The following studies were performed to exclude causes of secondary CVG&#58; plain x-rays of both hands&#44; cerebral computed tomography &#40;CT&#41;&#44; routine blood tests with thyroid function&#44; lipid profile&#44; insulin-like growth factor<span class="elsevierStyleHsp" style=""></span>1&#44; antinuclear antibodies&#44; and syphilis serology&#46; The cerebral CT of patient 1 showed the skin folds affecting the scalp &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Other studies were normal or negative&#46; Studies of the nail alterations &#40;culture and histopathology&#41; were only positive in patient 2 &#40;growth of <span class="elsevierStyleItalic">Trichophyton interdigitale</span>&#41;&#44; and treatment was prescribed with oral terbinafine &#40;250<span class="elsevierStyleHsp" style=""></span>mg&#47;24<span class="elsevierStyleHsp" style=""></span>h for 3 months&#41;&#46; Genetic analysis was not performed in either patient&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Primary essential CVG is most common in postpubertal men&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">1</span></a> The etiology is unknown&#44; but genetic and endocrine factors are believed to be implicated&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">2</span></a> The majority of cases of primary essential CVG are sporadic&#44; although familial forms with autosomal dominant or recessive inheritance with variable expression have been reported&#46;<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">3&#44;4</span></a> The prevalence data for the general population date from 1964&#44; with an estimated prevalence among men and women of 1 and 0&#46;026 cases per 100<span class="elsevierStyleHsp" style=""></span>000 population&#44; respectively&#46;<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&#44; in contrast to the primary nonessential form&#44; which can be associated with mental retardation&#44; cerebral palsy&#44; epilepsy&#44; schizophrenia&#44; deafness&#44; and cranial and ophthalmologic abnormalities&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">6</span></a> The secondary forms of CVG can be associated with numerous underlying diseases&#44; such as pachydermoperiostosis&#44; acromegaly&#44; cutis laxa&#44; cylindroma&#44; cerebriform intradermal nevus&#44; amyloidosis&#44; myxedema&#44; syphilis&#44; intracranial aneurysms&#44; intraventricular ependymoma&#44; and inflammatory conditions of the scalp&#59; exclusion of these associations is therefore important &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">7&#44;8</span></a></p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Primary CVG affects the scalp&#44; particularly at the vertex and in the occipital region&#46; The folds usually run symmetrically in an anterior-posterior direction&#44; although they can be transverse in the occipital region&#46; The number of folds varies between 2 and 12&#44; and they cannot be corrected by pressure or traction&#46;<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&#46; Imaging studies such as magnetic resonance imaging or CT must be performed when neurologic or ophthalmologic abnormalities or mental retardation are present&#44; although some authors recommend routinely performing these studies to exclude structural cerebral alterations&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">9</span></a> Scalp biopsy enables us to exclude other underlying causes&#44; such as cerebriform intradermal nevus&#44; cylindroma&#44; and inflammatory dermatoses of the scalp&#46; In the primary forms&#44; histopathology is normal in the majority of cases&#44; although thickening of the dermis and hypertrophy of the pilosebaceous units have been reported&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">10</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">CVG is a progressive disease and&#44; in its primary essential form&#44; only affects the scalp&#59; surgery is therefore only considered in cases with major psychological repercussions&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">11</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">In summary&#44; we have presented a case of familial primary essential CVG&#46; Primary essential CVG is a rare entity&#44; especially in its familial form&#44; with only 1 other familial case described&#44; also with clinical manifestation arising in adolescence&#46;<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&#46;</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&#46;</p></span></span>"
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Case and Research Letters
A Familial Form of Primary Essential Cutis Verticis Gyrata
Forma familiar de cutis verticis gyrata primario esencial
H. Suh
Autor para correspondencia
, Á. Flórez-Menéndez, C. de La Torre-Fraga
Servicio de Dermatología, Complejo Hospitalario Universitario de Pontevedra, Pontevedra, Spain
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    "titulo" => "A Familial Form of Primary Essential Cutis Verticis Gyrata"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A&#44; Anterior-posterior folding of the scalp &#40;patient<span class="elsevierStyleHsp" style=""></span>1&#41;&#46; B&#44; Anterior-posterior folding of the scalp &#40;patient<span class="elsevierStyleHsp" style=""></span>2&#41;&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Cutis verticis gyrata &#40;CVG&#41; is a rare skin condition characterized by thickening of the scalp&#44; giving rise to convolutions and sulci&#44; with a cerebriform pattern&#46; CVG is classified as primary &#40;essential and nonessential&#41; or secondary&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">We present 2 cases of primary essential CVG in a single family&#46; The 2 men &#40;patients 1 and 2&#41; were siblings of 32 and 28 years of age&#44; with no other relevant family history&#46; They presented progressive thickening of the scalp that had started in adolescence&#46; Physical examination revealed anterior-posterior folds on the scalp of both patients &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; and mild subungual hyperkeratosis and distal onycholysis of the nail plates of the great toes of both feet&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Detailed dermatologic and systemic medical history and physical examination&#44; including endocrine and neurologic evaluation&#44; detected none of the abnormalities associated with the primary nonessential forms&#46; Scalp biopsy revealed a mild nonspecific superficial periadnexal and perivascular mononuclear inflammatory infiltrate in both patients&#46; The following studies were performed to exclude causes of secondary CVG&#58; plain x-rays of both hands&#44; cerebral computed tomography &#40;CT&#41;&#44; routine blood tests with thyroid function&#44; lipid profile&#44; insulin-like growth factor<span class="elsevierStyleHsp" style=""></span>1&#44; antinuclear antibodies&#44; and syphilis serology&#46; The cerebral CT of patient 1 showed the skin folds affecting the scalp &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Other studies were normal or negative&#46; Studies of the nail alterations &#40;culture and histopathology&#41; were only positive in patient 2 &#40;growth of <span class="elsevierStyleItalic">Trichophyton interdigitale</span>&#41;&#44; and treatment was prescribed with oral terbinafine &#40;250<span class="elsevierStyleHsp" style=""></span>mg&#47;24<span class="elsevierStyleHsp" style=""></span>h for 3 months&#41;&#46; Genetic analysis was not performed in either patient&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Primary essential CVG is most common in postpubertal men&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">1</span></a> The etiology is unknown&#44; but genetic and endocrine factors are believed to be implicated&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">2</span></a> The majority of cases of primary essential CVG are sporadic&#44; although familial forms with autosomal dominant or recessive inheritance with variable expression have been reported&#46;<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">3&#44;4</span></a> The prevalence data for the general population date from 1964&#44; with an estimated prevalence among men and women of 1 and 0&#46;026 cases per 100<span class="elsevierStyleHsp" style=""></span>000 population&#44; respectively&#46;<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&#44; in contrast to the primary nonessential form&#44; which can be associated with mental retardation&#44; cerebral palsy&#44; epilepsy&#44; schizophrenia&#44; deafness&#44; and cranial and ophthalmologic abnormalities&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">6</span></a> The secondary forms of CVG can be associated with numerous underlying diseases&#44; such as pachydermoperiostosis&#44; acromegaly&#44; cutis laxa&#44; cylindroma&#44; cerebriform intradermal nevus&#44; amyloidosis&#44; myxedema&#44; syphilis&#44; intracranial aneurysms&#44; intraventricular ependymoma&#44; and inflammatory conditions of the scalp&#59; exclusion of these associations is therefore important &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">7&#44;8</span></a></p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Primary CVG affects the scalp&#44; particularly at the vertex and in the occipital region&#46; The folds usually run symmetrically in an anterior-posterior direction&#44; although they can be transverse in the occipital region&#46; The number of folds varies between 2 and 12&#44; and they cannot be corrected by pressure or traction&#46;<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&#46; Imaging studies such as magnetic resonance imaging or CT must be performed when neurologic or ophthalmologic abnormalities or mental retardation are present&#44; although some authors recommend routinely performing these studies to exclude structural cerebral alterations&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">9</span></a> Scalp biopsy enables us to exclude other underlying causes&#44; such as cerebriform intradermal nevus&#44; cylindroma&#44; and inflammatory dermatoses of the scalp&#46; In the primary forms&#44; histopathology is normal in the majority of cases&#44; although thickening of the dermis and hypertrophy of the pilosebaceous units have been reported&#46;<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">10</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">CVG is a progressive disease and&#44; in its primary essential form&#44; only affects the scalp&#59; surgery is therefore only considered in cases with major psychological repercussions&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">11</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">In summary&#44; we have presented a case of familial primary essential CVG&#46; Primary essential CVG is a rare entity&#44; especially in its familial form&#44; with only 1 other familial case described&#44; also with clinical manifestation arising in adolescence&#46;<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&#46;</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&#46;</p></span></span>"
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ISSN: 15782190
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