<?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>S2795-50012022000200165</article-id>
<article-id pub-id-type="doi">10.24875/pjd.m22000023</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Managing squamous cell carcinoma in recessive dystrophic epidermolysis bullosa with electrochemotherapy]]></article-title>
<article-title xml:lang="pt"><![CDATA[Eletroquimioterapia em carcinoma espinocelular na epidermólise bolhosa distrófica recessiva]]></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"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Brito-Caldeira]]></surname>
<given-names><![CDATA[Margarida]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Farricha]]></surname>
<given-names><![CDATA[Victor]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Carvalhal]]></surname>
<given-names><![CDATA[Sara]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Bartolo]]></surname>
<given-names><![CDATA[Joana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Moura]]></surname>
<given-names><![CDATA[Cecília]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Costa-Rosa]]></surname>
<given-names><![CDATA[Joaninha]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gouveia]]></surname>
<given-names><![CDATA[Emanuel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Nunes]]></surname>
<given-names><![CDATA[Hugo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Martins]]></surname>
<given-names><![CDATA[Mariluz]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Centro Hospitalar Universitário de Lisboa Central Dermatology and Venereology Department ]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Instituto Português de Oncologia de Lisboa Skin Cancer Multidisciplinary Group ]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>06</month>
<year>2022</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>06</month>
<year>2022</year>
</pub-date>
<volume>80</volume>
<numero>2</numero>
<fpage>165</fpage>
<lpage>168</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S2795-50012022000200165&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S2795-50012022000200165&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S2795-50012022000200165&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract Epidermolysis bullosa (EB) represents a group of congenital disorders caused by mutations in skin structural proteins. Squamous cell carcinoma (SCC) is one major complication and the leading cause of death of several subtypes of EB. We report a case of a 38-year-old female patient with recessive dystrophic EB that underwent electrochemotherapy (ECT) for treatment of cutaneous SCC in the right foot. Two sessions were performed, in which the intravenous administration of bleomycin was followed by the local application of electric pulses. We verified a partial response in 8th week of follow up and a complete response in the 24th week. ECT is a local treatment for cutaneous and subcutaneous tumors, mainly as palliative care for metastases. Studies have shown its effectiveness in primary tumors that are unresectable due to size and location, and it has been proposed as a new treatment modality for SCCs in EB with high overall response.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo A epidermólise bolhosa (EB) representa um grupo de doenças congénitas causadas por mutações em genes que codificam proteínas estruturais da pele. O carcinoma espinocelular (CEC) é uma das suas complicações mais temidas, sendo a principal causa de morte nalguns subtipos de EB. Apresenta-se o caso de uma doente de 38 anos com EB distrófica recessiva, que foi submetida a eletroquimioterapia (EQT) para o tratamento de um CEC no pé direito. Foram realizadas duas sessões de tratamento, com administração endovenosa de bleomicina seguida da aplicação de pulsos elétricos. Verificou-se uma resposta parcial às 8 semanas de follow-up e resposta completa às 24 semanas. A EQT é um tratamento local para tumores cutâneos e subcutâneos, primeiramente como modalidade paliativa em metástases. Estudos demonstram a sua eficácia no tratamento de tumores cutâneos primários irressecáveis, pelas suas dimensões ou localização, tendo sido proposta como modalidade terapêutica nos CECs na EB, com boa resposta global.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Recessive dystrophic epidermolysis bullosa]]></kwd>
<kwd lng="en"><![CDATA[Squamous cell carcinoma]]></kwd>
<kwd lng="en"><![CDATA[Electrochemotherapy]]></kwd>
<kwd lng="pt"><![CDATA[Epidermólise bolhosa distrófica recessiva]]></kwd>
<kwd lng="pt"><![CDATA[Carcinoma espinocelular]]></kwd>
<kwd lng="pt"><![CDATA[Eletroquimioterapia]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<label>1</label><nlm-citation citation-type="book">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Gedde-Dahl Jr]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Epidermolysis bullosa]]></article-title>
<source><![CDATA[A clinical, genetic and epidemiologic study]]></source>
<year>1971</year>
<page-range>1-180</page-range><publisher-loc><![CDATA[Baltimore ]]></publisher-loc>
<publisher-name><![CDATA[Johns Hopkins University Press]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B2">
<label>2</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Intong]]></surname>
<given-names><![CDATA[LR]]></given-names>
</name>
<name>
<surname><![CDATA[Murrell]]></surname>
<given-names><![CDATA[DF]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Inherited epidermolysis bullosa:new diagnostic criteria and classification]]></article-title>
<source><![CDATA[Clin Dermatol]]></source>
<year>2012</year>
<volume>30</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>70-7</page-range></nlm-citation>
</ref>
<ref id="B3">
<label>3</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Condorelli]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Dellambra]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Logli]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Zambruno]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Castiglia]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Epidermolysis bullosa-associated squamous cell carcinoma:from pathogenesis to therapeutic perspectives]]></article-title>
<source><![CDATA[Int J Mol Sci]]></source>
<year>2019</year>
<volume>20</volume>
<numero>22</numero>
<issue>22</issue>
<page-range>5707</page-range></nlm-citation>
</ref>
<ref id="B4">
<label>4</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Mellerio]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Robertson]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Bernardis]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Diem]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Fine]]></surname>
<given-names><![CDATA[JD]]></given-names>
</name>
<name>
<surname><![CDATA[George]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Management of cutaneous squamous cell carcinoma in patients with epidermolysis bullosa:best clinical practice guidelines]]></article-title>
<source><![CDATA[Br J Dermatol]]></source>
<year>2015</year>
<volume>174</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>56-67</page-range></nlm-citation>
</ref>
<ref id="B5">
<label>5</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Yamada]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Hatta]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Sogo]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Komura]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Hamaguchi]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Takehara]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Management of squamous cell carcinoma in a patient with recessive type epidermolysis bullosa dystrophica]]></article-title>
<source><![CDATA[Dermatol Surg]]></source>
<year>2004</year>
<volume>30</volume>
<page-range>424-1429</page-range></nlm-citation>
</ref>
<ref id="B6">
<label>6</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Bastin]]></surname>
<given-names><![CDATA[KT]]></given-names>
</name>
<name>
<surname><![CDATA[Steeves]]></surname>
<given-names><![CDATA[RA]]></given-names>
</name>
<name>
<surname><![CDATA[Richards]]></surname>
<given-names><![CDATA[MJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Radiation therapy for squamous cell carcinoma in dystrophic epidermolysis bullosa:case reports and literature review]]></article-title>
<source><![CDATA[Am J Clin Oncol]]></source>
<year>1997</year>
<volume>20</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>55-8</page-range></nlm-citation>
</ref>
<ref id="B7">
<label>7</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lentz]]></surname>
<given-names><![CDATA[SR]]></given-names>
</name>
<name>
<surname><![CDATA[Raish]]></surname>
<given-names><![CDATA[RJ]]></given-names>
</name>
<name>
<surname><![CDATA[Orlowski]]></surname>
<given-names><![CDATA[EP]]></given-names>
</name>
<name>
<surname><![CDATA[Marion]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Squamous cell carcinoma in epidermolysis bullosa:treatment with systemic chemotherapy]]></article-title>
<source><![CDATA[Cancer]]></source>
<year>1990</year>
<volume>66</volume>
<page-range>1276-8</page-range></nlm-citation>
</ref>
<ref id="B8">
<label>8</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Mir]]></surname>
<given-names><![CDATA[LM]]></given-names>
</name>
<name>
<surname><![CDATA[Belehradek]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Domenge]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Orlowski]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Poddevin]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Belehradek Jr]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Electrochemotherapy, a new antitumour treatment:first clinical trial]]></article-title>
<source><![CDATA[C R Acad Sci III]]></source>
<year>1991</year>
<volume>313</volume>
<numero>13</numero>
<issue>13</issue>
<page-range>613-8</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Mir]]></surname>
<given-names><![CDATA[LM]]></given-names>
</name>
<name>
<surname><![CDATA[Gehl]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Sersa]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Collins]]></surname>
<given-names><![CDATA[CG]]></given-names>
</name>
<name>
<surname><![CDATA[Garbay]]></surname>
<given-names><![CDATA[JR]]></given-names>
</name>
<name>
<surname><![CDATA[Billard]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Standard operating procedures of the electrochemotherapy:instructions for the use of bleomycin or cisplatin administered either systemically or locally and electric pulses delivered by the Cliniporator by means of invasive or non-invasive electrodes]]></article-title>
<source><![CDATA[Eur J Cancer]]></source>
<year>2006</year>
<volume>4</volume>
<page-range>14-25</page-range></nlm-citation>
</ref>
<ref id="B10">
<label>10</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Mali]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Jarm]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Snoj]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Sersa]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Miklavcic]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Antitumor effectiveness of electrochemotherapy:a systematic review and meta-analysis]]></article-title>
<source><![CDATA[Eur J Surg Oncol]]></source>
<year>2013</year>
<volume>39</volume>
<page-range>4-16</page-range></nlm-citation>
</ref>
<ref id="B11">
<label>11</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Mir]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Glass]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Ser&#353;a]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Teissié]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Domenge]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Miklav&#269;i&#269;]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Effective treatment of cutaneous and subcutaneous malignant tumours by electrochemotherapy]]></article-title>
<source><![CDATA[Br J Cancer]]></source>
<year>1998</year>
<volume>77</volume>
<numero>12</numero>
<issue>12</issue>
<page-range>2336-42</page-range></nlm-citation>
</ref>
<ref id="B12">
<label>12</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Diociaiuti]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Rotunno]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[el Hachem]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Latorre]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Cozza]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Curatolo]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Electrochemotherapy, a potential new treatment for the management of squamous cell carcinoma in patients with recessive dystrophic epidermolysis bullosa:report of three cases]]></article-title>
<source><![CDATA[J Eur Acad Dermatol Venereol]]></source>
<year>2015</year>
<volume>30</volume>
<numero>7</numero>
<issue>7</issue>
<page-range>1195-6</page-range></nlm-citation>
</ref>
<ref id="B13">
<label>13</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Bartolo]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Farricha]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Carvalhal]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Moura]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Abecasis]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Electrochemotherapy, a local treatment for squamous cell carcinoma in patients with recessive dystrophic epidermolysis bullosa]]></article-title>
<source><![CDATA[Dermatol Ther]]></source>
<year>2020</year>
<volume>33</volume>
<numero>6</numero>
<issue>6</issue>
</nlm-citation>
</ref>
<ref id="B14">
<label>14</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Therasse]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Arbuck]]></surname>
<given-names><![CDATA[SG]]></given-names>
</name>
<name>
<surname><![CDATA[Eisenhauer]]></surname>
<given-names><![CDATA[EA]]></given-names>
</name>
<name>
<surname><![CDATA[Wanders]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Kaplan]]></surname>
<given-names><![CDATA[RS]]></given-names>
</name>
<name>
<surname><![CDATA[Rubinstein]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[New guidelines to evaluate the response to treatment in solid tumours. European Organization for Research and Treatment of Cancer, National Cancer Institute of the United States, National Cancer Institute of Canada]]></article-title>
<source><![CDATA[J Natl Cancer Inst]]></source>
<year>2000</year>
<volume>92</volume>
<page-range>205-16</page-range></nlm-citation>
</ref>
<ref id="B15">
<label>15</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Fine]]></surname>
<given-names><![CDATA[JD]]></given-names>
</name>
<name>
<surname><![CDATA[Mellerio]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Extracutaneous manifestations and complications of inherited epidermolysis bullosa. Parts I&amp;II]]></article-title>
<source><![CDATA[J Am Acad Dermatol]]></source>
<year>2009</year>
<volume>61</volume>
<page-range>367-402</page-range></nlm-citation>
</ref>
<ref id="B16">
<label>16</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Fine]]></surname>
<given-names><![CDATA[JD]]></given-names>
</name>
<name>
<surname><![CDATA[Johnson]]></surname>
<given-names><![CDATA[LB]]></given-names>
</name>
<name>
<surname><![CDATA[Weiner]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Li]]></surname>
<given-names><![CDATA[KP]]></given-names>
</name>
<name>
<surname><![CDATA[Suchindran]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Epidermolysis bullosa and the risk of life-threatening cancers:national EB registry experience, 1986&#8211;2006]]></article-title>
<source><![CDATA[J Am Acad Dermatol]]></source>
<year>2009</year>
<volume>60</volume>
<page-range>203-11</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
