<?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>2795-5001</journal-id>
<journal-title><![CDATA[Portuguese Journal of Dermatology and Venereology]]></journal-title>
<abbrev-journal-title><![CDATA[Port J Dermatol Venereol.]]></abbrev-journal-title>
<issn>2795-5001</issn>
<publisher>
<publisher-name><![CDATA[Permanyer Publications]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2795-50012025000200124</article-id>
<article-id pub-id-type="doi">10.24875/pjdv.24000087</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Cutaneous IgG4-related disease treated with dupilumab]]></article-title>
<article-title xml:lang="pt"><![CDATA[Doença cutânea relacionada com IgG4 tratada com dupilumab]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Pestana]]></surname>
<given-names><![CDATA[Mafalda]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[João]]></surname>
<given-names><![CDATA[Alexandre]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Palma-Carlos]]></surname>
<given-names><![CDATA[Susana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Leiria-Pinto]]></surname>
<given-names><![CDATA[Paula]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[João]]></surname>
<given-names><![CDATA[Ana L.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Unidade de Saúde Local de São José Department of Dermatology and Venereology ]]></institution>
<addr-line><![CDATA[Lisbon ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Centro Clínico Académico de Lisboa Centro Clínico Académico de Lisboa ]]></institution>
<addr-line><![CDATA[Lisbon ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Unidade de Saúde Local de São José Department of Immunoallergology ]]></institution>
<addr-line><![CDATA[Lisbon ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>06</month>
<year>2025</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>06</month>
<year>2025</year>
</pub-date>
<volume>83</volume>
<numero>2</numero>
<fpage>124</fpage>
<lpage>128</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S2795-50012025000200124&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S2795-50012025000200124&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S2795-50012025000200124&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract We present a 59-year-old male with a prolonged history of severe, treatment-resistant pruritic dermatosis and associated systemic symptoms, including fatigue and diarrhea. Dermatologic examination revealed widespread erythematous-brownish papules and nodules, prompting a skin biopsy that showed dense infiltration by immunoglobulin G4 (IgG4)-positive plasma cells, leading to a diagnosis of IgG4-related disease (IgG4-RD). The patient was treated with dupilumab, resulting in complete skin lesion resolution and significant improvement in quality of life. IgG4-RD, a rare inflammatory disease with potential multiorgan involvement, frequently challenges diagnosis due to diverse clinical presentations. This case highlights dupilumab effectiveness as a novel therapy for IgG4-RD with cutaneous involvement, offering a promising alternative for patients who do not respond well to corticosteroids.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo Reportamos o caso de um homem de 59 anos, com dermatose intensamente pruriginosa desde há 7 anos, refratária à terapêutica, e associada a sintomatologia sistémica como astenia e diarreia. Ao exame objetivo dermatológico, o doente apresentava pápulo-nódulos eritemato-acastanhados disseminados, o que motivou a realização de biópsia cutânea cujo exame histopatológico revelou infiltrado rico em plasmócitos IgG4-positivo. Admitido o diagnóstico de doença relacionada com IgG4 (DR-IgG4), o doente iniciou tratamento com dupilumab, com consequente resolução da dermatose e melhoria franca da sua qualidade de vida. A DR-IgG4, uma patologia inflamatória rara com potencial envolvimento multiorgânico, representa frequentemente um desafio diagnóstico pela sua hetereogeneidade clínica. Este caso clínico enaltece a eficácia do dupilumab na DR-IgG4 com envolvimento cutâneo, surgindo como uma alternativa terapêutica promissora na doença não respondedora a corticoterapia.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Immunoglobulin G4-related disease]]></kwd>
<kwd lng="en"><![CDATA[Cutaneous]]></kwd>
<kwd lng="en"><![CDATA[Dupilumab]]></kwd>
<kwd lng="pt"><![CDATA[Doença relacionada com IgG4-related]]></kwd>
<kwd lng="pt"><![CDATA[Cutânea]]></kwd>
<kwd lng="pt"><![CDATA[Dupilumab]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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