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e seis mulheres&#44; todas afetadas pela doen&#231;a &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a>&#41;&#46; Os pais n&#227;o eram consangu&#237;neos e n&#227;o foram examinados&#44; pois j&#225; faleceram&#46; As irm&#227;s moram na mesma cidade e n&#227;o vivem juntas&#46; Todas s&#227;o menopausadas e nenhuma recebeu terapia de reposi&#231;&#227;o hormonal &#40;TRH&#41;&#46; A idade de in&#237;cio da AFF variou de&#160;31&#160;a&#160;62&#160;anos e a idade m&#233;dia foi de&#160;50&#44;7&#160;anos&#46; Cinco t&#234;m alopecia de sobrancelhas e c&#237;lios e quatro t&#234;m perda de pelos no corpo&#46; O l&#237;quen plano pigmentoso &#40;LPPig&#41; &#233; observado em duas irm&#227;s e p&#225;pulas faciais em tr&#234;s&#46; O diagn&#243;stico foi feito pelas caracter&#237;sticas cl&#237;nicas e tricosc&#243;picas &#40;<a class="elsevierStyleCrossRef" href="#fig0010">fig&#46; 2</a>&#41;&#44; pois elas se recusaram a se submeter &#224; bi&#243;psia&#46; Como diagn&#243;stico diferencial&#44; poder&#8208;se&#8208;ia pensar em ceratose pilar atr&#243;fica&#44; mas nenhuma delas apresentava ceratose pilar&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">De comorbidades encontramos hipertens&#227;o arterial sist&#234;mica &#40;HAS&#41; e diabetes <span class="elsevierStyleItalic">mellitus</span> &#40;DM&#41; em uma paciente&#44; trombose venosa profunda &#40;TVP&#41; e rinite al&#233;rgica em outras duas&#46; N&#227;o h&#225; casos de disfun&#231;&#227;o tireoidiana&#46; Os dados cl&#237;nicos e demogr&#225;ficos est&#227;o resumidos na <a class="elsevierStyleCrossRef" href="#tbl0005">tabela 1</a>&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Os padr&#245;es cl&#237;nicos de AFF t&#234;m sido descritos com base na recess&#227;o frontal do cabelo como padr&#227;o&#160;I &#40;linear&#41;&#44; padr&#227;o&#160;II &#40;difuso&#41;&#44; padr&#227;o&#160;III &#40;pseudo&#8208;&#8220;sinal da franja&#8221;&#41;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> e padr&#227;o irregular&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> Em nossa s&#233;rie&#44; o padr&#227;o II &#233; encontrado em todas as pacientes e em uma&#44; o padr&#227;o irregular&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">A gen&#233;tica da AFF ainda &#233; desconhecida&#46; Estudos mostram que o HLA&#8208;DR1 foi implicado no l&#237;quen plano e na s&#237;ndrome de Graham&#8208;Little&#8208;Piccardi&#8208;Lassueur&#44; mas n&#227;o foi encontrado em AFF&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Casos familiares podem indicar um mecanismo gen&#233;tico&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Al&#233;m disso&#44; a epidemia de AFF observada atualmente sugere fortemente que um gatilho ambiental tamb&#233;m pode estar envolvido&#46; Nesta s&#233;rie&#44; n&#227;o pudemos definir qualquer subst&#226;ncia que pudesse ser apontada como esse fator&#44; apesar de todas terem usado protetor solar em algum momento de suas vidas&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">At&#233; onde sabemos&#44; esta &#233; a maior s&#233;rie com AFF na mesma fam&#237;lia j&#225; descrita&#46; Estudar essas fam&#237;lias pode ajudar a entender a gen&#233;tica e a patog&#234;nese dessa intrigante doen&#231;a&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Suporte financeiro</span><p id="par0040" class="elsevierStylePara elsevierViewall">Nenhum&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Contribui&#231;&#227;o dos autores</span><p id="par0045" class="elsevierStylePara elsevierViewall">Vanessa Barreto Rocha&#58; Aprova&#231;&#227;o da vers&#227;o final do manuscrito&#59; concep&#231;&#227;o e planejamento do estudo&#59; elabora&#231;&#227;o e reda&#231;&#227;o do manuscrito&#59; obten&#231;&#227;o&#44; an&#225;lise e interpreta&#231;&#227;o dos dados&#59; revis&#227;o cr&#237;tica da literatura&#59; revis&#227;o cr&#237;tica do manuscrito&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Mario Cezar Pires&#58; Aprova&#231;&#227;o da vers&#227;o final do manuscrito&#59; participa&#231;&#227;o efetiva na orienta&#231;&#227;o da pesquisa&#59; revis&#227;o cr&#237;tica do manuscrito&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Leticia Arsie Contin&#58; Aprova&#231;&#227;o da vers&#227;o final do manuscrito&#59; concep&#231;&#227;o e planejamento do estudo&#59; elabora&#231;&#227;o e reda&#231;&#227;o do manuscrito&#59; obten&#231;&#227;o&#44; an&#225;lise e interpreta&#231;&#227;o dos dados&#59; participa&#231;&#227;o efetiva na orienta&#231;&#227;o da pesquisa&#59; participa&#231;&#227;o intelectual em conduta proped&#234;utica e&#47;ou terap&#234;utica de casos estudados&#59; 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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Alopecia c&#237;lios&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">P&#225;pulas faciais&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Idade da menopausa&#47; TRH&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Comorbidades&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">67a&#44; III&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">52a&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">II &#40;difusa&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Sim&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Sim&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Sim&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Sim&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">N&#227;o&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">55a &#47; N&#227;o&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">64a&#44; V&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">62a&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">Placas<span class="elsevierStyleHsp" style=""></span>&#43;<span class="elsevierStyleHsp" style=""></span>II&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">N&#227;o&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Sim&#44; parcial&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">48a&#47;N&#227;o &#40;histerectomia&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">N&#227;o&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">Sim&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Sim&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">N&#227;o&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">55a&#47;N&#227;o&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">II&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">N&#227;o&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">Sim&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">N&#227;o&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Sim&nbsp;\t\t\t\t\t\t\n
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Carta ‐ Caso clínico
Alopecia frontal fibrosante familiar em seis irmãs
Vanessa Barreto Rocha
Corresponding author
vanessabarreto@oi.com.br

Autor para correspondência.
, Mario Cezar Pires, Leticia Arsie Contin
Instituto de Assistência Médica ao Servidor Público Estadual, Vila Clementino, SP, Brasil
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    "titulo" => "Alopecia frontal fibrosante familiar em seis irm&#227;s"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A alopecia frontal fibrosante &#40;AFF&#41; &#233; uma alopecia cicatricial progressiva descrita pela primeira vez por Kossard em&#160;1994 e que tem se tornado mundialmente epid&#234;mica na &#250;ltima d&#233;cada&#46; Casos familiares de AFF v&#234;m sendo descritos desde 2008&#44;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> mas as s&#233;ries incluem at&#233; tr&#234;s parentes na mesma gera&#231;&#227;o ou cinco em duas gera&#231;&#245;es&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2&#44;3</span></a> Encontramos um grupo de seis irm&#227;s que apresentavam diagn&#243;stico cl&#237;nico e histol&#243;gico de AFF no Brasil&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Essa fam&#237;lia &#233; composta por oito irm&#227;os&#44; dois homens &#40;n&#227;o examinados&#41; e seis mulheres&#44; todas afetadas pela doen&#231;a &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a>&#41;&#46; Os pais n&#227;o eram consangu&#237;neos e n&#227;o foram examinados&#44; pois j&#225; faleceram&#46; As irm&#227;s moram na mesma cidade e n&#227;o vivem juntas&#46; Todas s&#227;o menopausadas e nenhuma recebeu terapia de reposi&#231;&#227;o hormonal &#40;TRH&#41;&#46; A idade de in&#237;cio da AFF variou de&#160;31&#160;a&#160;62&#160;anos e a idade m&#233;dia foi de&#160;50&#44;7&#160;anos&#46; Cinco t&#234;m alopecia de sobrancelhas e c&#237;lios e quatro t&#234;m perda de pelos no corpo&#46; O l&#237;quen plano pigmentoso &#40;LPPig&#41; &#233; observado em duas irm&#227;s e p&#225;pulas faciais em tr&#234;s&#46; O diagn&#243;stico foi feito pelas caracter&#237;sticas cl&#237;nicas e tricosc&#243;picas &#40;<a class="elsevierStyleCrossRef" href="#fig0010">fig&#46; 2</a>&#41;&#44; pois elas se recusaram a se submeter &#224; bi&#243;psia&#46; Como diagn&#243;stico diferencial&#44; poder&#8208;se&#8208;ia pensar em ceratose pilar atr&#243;fica&#44; mas nenhuma delas apresentava ceratose pilar&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">De comorbidades encontramos hipertens&#227;o arterial sist&#234;mica &#40;HAS&#41; e diabetes <span class="elsevierStyleItalic">mellitus</span> &#40;DM&#41; em uma paciente&#44; trombose venosa profunda &#40;TVP&#41; e rinite al&#233;rgica em outras duas&#46; N&#227;o h&#225; casos de disfun&#231;&#227;o tireoidiana&#46; Os dados cl&#237;nicos e demogr&#225;ficos est&#227;o resumidos na <a class="elsevierStyleCrossRef" href="#tbl0005">tabela 1</a>&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Os padr&#245;es cl&#237;nicos de AFF t&#234;m sido descritos com base na recess&#227;o frontal do cabelo como padr&#227;o&#160;I &#40;linear&#41;&#44; padr&#227;o&#160;II &#40;difuso&#41;&#44; padr&#227;o&#160;III &#40;pseudo&#8208;&#8220;sinal da franja&#8221;&#41;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> e padr&#227;o irregular&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> Em nossa s&#233;rie&#44; o padr&#227;o II &#233; encontrado em todas as pacientes e em uma&#44; o padr&#227;o irregular&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">A gen&#233;tica da AFF ainda &#233; desconhecida&#46; Estudos mostram que o HLA&#8208;DR1 foi implicado no l&#237;quen plano e na s&#237;ndrome de Graham&#8208;Little&#8208;Piccardi&#8208;Lassueur&#44; mas n&#227;o foi encontrado em AFF&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Casos familiares podem indicar um mecanismo gen&#233;tico&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Al&#233;m disso&#44; a epidemia de AFF observada atualmente sugere fortemente que um gatilho ambiental tamb&#233;m pode estar envolvido&#46; Nesta s&#233;rie&#44; n&#227;o pudemos definir qualquer subst&#226;ncia que pudesse ser apontada como esse fator&#44; apesar de todas terem usado protetor solar em algum momento de suas vidas&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">At&#233; onde sabemos&#44; esta &#233; a maior s&#233;rie com AFF na mesma fam&#237;lia j&#225; descrita&#46; Estudar essas fam&#237;lias pode ajudar a entender a gen&#233;tica e a patog&#234;nese dessa intrigante doen&#231;a&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Suporte financeiro</span><p id="par0040" class="elsevierStylePara elsevierViewall">Nenhum&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Contribui&#231;&#227;o dos autores</span><p id="par0045" class="elsevierStylePara elsevierViewall">Vanessa Barreto Rocha&#58; Aprova&#231;&#227;o da vers&#227;o final do manuscrito&#59; concep&#231;&#227;o e planejamento do estudo&#59; elabora&#231;&#227;o e reda&#231;&#227;o do manuscrito&#59; obten&#231;&#227;o&#44; an&#225;lise e interpreta&#231;&#227;o dos dados&#59; revis&#227;o cr&#237;tica da literatura&#59; revis&#227;o cr&#237;tica do manuscrito&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Mario Cezar Pires&#58; Aprova&#231;&#227;o da vers&#227;o final do manuscrito&#59; participa&#231;&#227;o efetiva na orienta&#231;&#227;o da pesquisa&#59; revis&#227;o cr&#237;tica do manuscrito&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Leticia Arsie Contin&#58; Aprova&#231;&#227;o da vers&#227;o final do manuscrito&#59; concep&#231;&#227;o e planejamento do estudo&#59; elabora&#231;&#227;o e reda&#231;&#227;o do manuscrito&#59; obten&#231;&#227;o&#44; an&#225;lise e interpreta&#231;&#227;o dos dados&#59; participa&#231;&#227;o efetiva na orienta&#231;&#227;o da pesquisa&#59; participa&#231;&#227;o intelectual em conduta proped&#234;utica e&#47;ou terap&#234;utica de casos estudados&#59; 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          "pt" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Dados cl&#237;nicos e demogr&#225;ficos de seis irm&#227;s com alopecia frontal fibrosante</p>"
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Article information
ISSN: 26662752
Original language: Portuguese
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