<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>2182-2395</journal-id>
<journal-title><![CDATA[Revista da Sociedade Portuguesa de Dermatologia e Venereologia]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Soc Port Dermatol Venereol]]></abbrev-journal-title>
<issn>2182-2395</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Dermatologia e Venereologia]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2182-23952021000400057</article-id>
<article-id pub-id-type="doi">10.29021/spdv.79.4.1385</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Purpura Fulminans in a 20-Year-Old Female]]></article-title>
<article-title xml:lang="pt"><![CDATA[Púrpura Fulminante numa Jovem de 20 Anos]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Alves]]></surname>
<given-names><![CDATA[Francisca]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Brites]]></surname>
<given-names><![CDATA[Maria Manuel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Coutinho]]></surname>
<given-names><![CDATA[Inês]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Centro Hospitalar Universitário de Coimbra Serviço de Dermatologia ]]></institution>
<addr-line><![CDATA[Coimbra ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>30</day>
<month>12</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>30</day>
<month>12</month>
<year>2021</year>
</pub-date>
<volume>79</volume>
<numero>4</numero>
<fpage>57</fpage>
<lpage>59</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S2182-23952021000400057&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S2182-23952021000400057&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S2182-23952021000400057&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT A previously healthy 20-year-old female presented with extensive retiform purpura located at the face, upper and lower limbs, one week after an episode of acute tonsillitis. Despite the exuberance of the cutaneous findings and progression to skin necrosis she had no accompanying symptoms. Laboratory investigation revealed a heterozygous protein C mutation (exon 9, c.1332G&gt; C, p.Trp444Cys), accounting for a partial deficiency of this anticoagulant protein. The patient was started on broad spectrum antibiotics, anticoagulation and systemic corticosteroids, with no lesional progression and complete resolution of cutaneous ulceration within 6 months. This is a singular case of purpura fulminans, since two different causative factors precipitated the events. The previous tonsillitis reported by the patient is significant, because the serum concentration of protein S may also decrease after an infectious event - post-infectious purpura fulminans. This case illustrates that purpura fulminans due to autoantibodies against protein S, although rare, should be considered, especially in the absence of a severe acute infection. It also illustrates how in a given patient different independent factors can act simultaneously, triggering potentially devastating clinical scenarios.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[RESUMO Apresentamos o caso de uma jovem do sexo feminino, de 20 anos de idade, previamente saudável, que se apresentou com lesões muito extensas de púrpura retiforme localizadas na face, membros superiores e inferiores, uma semana após um episódio de amigdalite aguda. Apesar das lesões cutâneas exuberantes que evoluiram para necrose cutânea, a doente não apresentava qualquer sintomatologia associada. A investigação laboratorial revelou uma mutação em heterozigotia da proteína C (exon 9, c.1332G&gt; C, p.Trp444Cys), condicionando um deficit parcial desta proteína anticoagulante. Iniciou antibioticoterapia de largo espectro, anticoagulação e corticoterapia sistémica, sem progressão das lesões no imediato, e resolução completa das ulcerações em 6 meses. Este é um caso singular de púrpura fulminante, visto que que dois fatores etiológicos diferentes precipitaram os eventos. A amigdalite prévia rela-tada pela doente é significativa, pois a concentração sérica da proteína S também pode diminuir após um evento infeccioso - purpura fulminans pós-infecciosa. Este caso ilustra que a púrpura fulminante decorrente de auto-anticorpos contra a proteína S, embora rara, deve ser considerada, principalmente na ausência de infecção aguda grave. Também evidencia como, em um determinado doente, diferentes fatores independentes podem atuar simultaneamente, desencadeando cenários clínicos potencialmente devastadores.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Protein C Deficiency]]></kwd>
<kwd lng="en"><![CDATA[Protein S]]></kwd>
<kwd lng="en"><![CDATA[Purpura Fulminans.]]></kwd>
<kwd lng="pt"><![CDATA[Deficiência de Proteína C]]></kwd>
<kwd lng="pt"><![CDATA[Proteína S]]></kwd>
<kwd lng="pt"><![CDATA[Púrpura Fulminante.]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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