<?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>0121-0319</journal-id>
<journal-title><![CDATA[Medicas UIS]]></journal-title>
<abbrev-journal-title><![CDATA[Medicas UIS]]></abbrev-journal-title>
<issn>0121-0319</issn>
<publisher>
<publisher-name><![CDATA[Universidad Industrial de Santander]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0121-03192021000100010</article-id>
<article-id pub-id-type="doi">10.18273/revmed.v34n1-2021010</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Pénfigo benigno familiar de presentación atípica en paciente sin antecedente familiar]]></article-title>
<article-title xml:lang="en"><![CDATA[Atypical presentation of familial benign pemphigus in patient without familiar background]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Chaustre Torres]]></surname>
<given-names><![CDATA[Linda Adori]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Torra Barajas]]></surname>
<given-names><![CDATA[Oscar Leonardo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Rubiano]]></surname>
<given-names><![CDATA[Cesar Augusto]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gutiérrez Moreno]]></surname>
<given-names><![CDATA[Mario Fernando]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Médico residente de primer año. Posgrado de Dermatología. Universidad de Manizales  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Médico general. Universidad Autónoma Bucaramanga  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Médico general. Universidad Autónoma Bucaramanga  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Médico general Universidad De Pamplona  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Colombia</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>04</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>04</month>
<year>2021</year>
</pub-date>
<volume>34</volume>
<numero>1</numero>
<fpage>101</fpage>
<lpage>106</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_arttext&amp;pid=S0121-03192021000100010&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_abstract&amp;pid=S0121-03192021000100010&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_pdf&amp;pid=S0121-03192021000100010&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen La enfermedad de Hailey-Hailey, también llamada pénfigo familiar benigno, corresponde a una genodermatosis debilitante que se transmite mediante un patrón autosómico dominante, con una prevalencia de alrededor de 1 en 50.000 casos. El reporte de antecedentes familiares está presente hasta en 60 % de los pacientes. Se caracteriza por la presencia de vesículas crónicas y recurrentes, erosiones y exulceraciones en zonas de flexura. El tratamiento puede representar un reto, porque a pesar del manejo con terapias tópicas, corticosteroides sistémicos, inmunomoduladores sistémicos y el empleo de láser, ninguna terapia ha logrado una remisión a largo plazo. Se presenta el caso de un paciente masculino, adulto medio, sin antecedente familiar alguno, con historia de placas de superficie descamativa y hematocostras recurrentes crónicas y presentación clínica atípica, dada la localización de lesiones predominantes en miembros superiores, con sospecha inicial de psoriasis vulgar, con posterior toma de biopsia y reporte de patología que evidencia histológia típica de PBF. Por lo cual se indica manejo con corticosteroides sistémicos, sin evidencia de reacciones adversas y con remisión a largo plazo. MÉD.UIS.2020;34(1):101-6]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract Hailey-Hailey disease, also called benign familial pemphigus, corresponds to a debilitating genodermatosis that is transmitted through an autosomal dominant pattern, with a prevalence of around 1 in 50,000 cases. The family history report is present in up to 60% of patients. It is characterized by the presence of chronic and recurrent vesicles, erosions and exulcerations in flexural areas. Treatment can be challenging, because despite management with topical therapies, systemic corticosteroids, systemic immunomodulators, and the use of lasers, no therapy has achieved long-term remission.We present the case of a male patient, middle adult, without any family history, with a history of scaly surface plaques and chronic recurrent hematocostras and atypical clinical presentation given the location of predominant lesions in the upper limbs, with initial suspicion of vulgar psoriasis, with subsequent biopsy and pathology report showing typical PBF histology. Therefore, management with systemic corticosteroids without evidence of adverse reactions and with long-term remission is indicated. MÉD.UIS.2020;34(1):101-6]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Pénfigo Familiar Benigno]]></kwd>
<kwd lng="es"><![CDATA[Enfermedad de Hailey-Hailey]]></kwd>
<kwd lng="es"><![CDATA[Acantolisis]]></kwd>
<kwd lng="es"><![CDATA[Tratamiento]]></kwd>
<kwd lng="es"><![CDATA[Corticoesteroides]]></kwd>
<kwd lng="en"><![CDATA[Familial benign pemphigus]]></kwd>
<kwd lng="en"><![CDATA[Hailey-Hailey disease]]></kwd>
<kwd lng="en"><![CDATA[Acantholysis]]></kwd>
<kwd lng="en"><![CDATA[Treatment]]></kwd>
<kwd lng="en"><![CDATA[Corticosteroids]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<label>1.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lamb]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Benign familial pemphigus]]></article-title>
<source><![CDATA[DermNet NZ]]></source>
<year>2016</year>
</nlm-citation>
</ref>
<ref id="B2">
<label>2.</label><nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Benign chronic pemphigus. Genetics Home Reference]]></article-title>
<source><![CDATA[Medlineplus.gov]]></source>
<year>2016</year>
</nlm-citation>
</ref>
<ref id="B3">
<label>3.</label><nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Hailey-Hailey disease]]></article-title>
<source><![CDATA[NORD]]></source>
<year>2015</year>
</nlm-citation>
</ref>
<ref id="B4">
<label>4.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Helm]]></surname>
<given-names><![CDATA[TN]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Familial Benign Pemphigus (Hailey-Hailey Disease)]]></article-title>
<source><![CDATA[Medscape Reference]]></source>
<year>2017</year>
</nlm-citation>
</ref>
<ref id="B5">
<label>5.</label><nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Familial benign chronic pemphigus]]></article-title>
<source><![CDATA[Orphanet]]></source>
<year>2006</year>
</nlm-citation>
</ref>
<ref id="B6">
<label>6.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Farahnik]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Blattner]]></surname>
<given-names><![CDATA[CM]]></given-names>
</name>
<name>
<surname><![CDATA[Mortazie]]></surname>
<given-names><![CDATA[MB]]></given-names>
</name>
<name>
<surname><![CDATA[Perry]]></surname>
<given-names><![CDATA[BM]]></given-names>
</name>
<name>
<surname><![CDATA[Lear]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
<name>
<surname><![CDATA[Elston]]></surname>
<given-names><![CDATA[DM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Interventional treatments for Hailey-Hailey disease]]></article-title>
<source><![CDATA[J Am Acad Dermatol]]></source>
<year>2017</year>
<volume>76</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>551-8</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[Prateek]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Banwarilal]]></surname>
<given-names><![CDATA[MR]]></given-names>
</name>
<name>
<surname><![CDATA[Chaudhary]]></surname>
<given-names><![CDATA[SS]]></given-names>
</name>
<name>
<surname><![CDATA[Garg]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hailey Hailey disease-a rare case report]]></article-title>
<source><![CDATA[Int J Res Dermatol]]></source>
<year>2016</year>
<volume>2</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>36-9</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[Medina]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Moreno]]></surname>
<given-names><![CDATA[JC]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Enfermedad de Hailey-Hailey]]></article-title>
<source><![CDATA[Piel]]></source>
<year>2001</year>
<volume>16</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>272-7</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9.</label><nlm-citation citation-type="journal">
<collab>British Association of Dermatologists</collab>
<article-title xml:lang=""><![CDATA[Patient leaflet]]></article-title>
<source><![CDATA[British Association of Dermatologists]]></source>
<year>2014</year>
</nlm-citation>
</ref>
<ref id="B10">
<label>10.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Rodríguez]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Ramos]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Sánchez]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Pénfigo benigno familiar (Enfermedad de Hailey-Hailey)]]></article-title>
<source><![CDATA[Rev Cent Dermatol Pascua]]></source>
<year>2009</year>
<volume>18</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>25-30</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[Ibrahim]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
<name>
<surname><![CDATA[Hogan]]></surname>
<given-names><![CDATA[SR]]></given-names>
</name>
<name>
<surname><![CDATA[Vij]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Fernandez]]></surname>
<given-names><![CDATA[AP]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Naltrexone Treatment of Familial Benign Pemphigus (Hailey-Hailey Disease)]]></article-title>
<source><![CDATA[JAMA Dermatol]]></source>
<year>2017</year>
<volume>153</volume>
<numero>10</numero>
<issue>10</issue>
<page-range>1015-7</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[Arora]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Bray]]></surname>
<given-names><![CDATA[FN]]></given-names>
</name>
<name>
<surname><![CDATA[Cervantes]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Falto-Aizpurua]]></surname>
<given-names><![CDATA[LA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Management of familial benign chronic pemphigus]]></article-title>
<source><![CDATA[Clin Cosmet Investig Dermatol]]></source>
<year>2016</year>
<volume>9</volume>
<page-range>281-90</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[Biocalti]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Aurizi]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Barbieri]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Cialfi]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Screnpanti]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Talora]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Efficacy of the melanocortin analogue Nle4-D-Phe7-&#945;-melanocyte-stimulating hormone in the treatment of patients with Hailey-Hailey disease]]></article-title>
<source><![CDATA[Clin Exp Dermatol]]></source>
<year>2014</year>
<volume>39</volume>
<page-range>168-74</page-range></nlm-citation>
</ref>
<ref id="B14">
<label>14.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[White]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Shvartsbeyn]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Meehan]]></surname>
<given-names><![CDATA[SA]]></given-names>
</name>
<name>
<surname><![CDATA[Urbanek]]></surname>
<given-names><![CDATA[RW]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Benign familial pemphigus (Hailey&#8211;Hailey disease)]]></article-title>
<source><![CDATA[Dermatol Online J]]></source>
<year>2015</year>
<volume>21</volume>
<numero>12</numero>
<issue>12</issue>
</nlm-citation>
</ref>
<ref id="B15">
<label>15.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Grönemeyer]]></surname>
<given-names><![CDATA[LL]]></given-names>
</name>
<name>
<surname><![CDATA[Thoms]]></surname>
<given-names><![CDATA[KM]]></given-names>
</name>
<name>
<surname><![CDATA[Bertsch]]></surname>
<given-names><![CDATA[HP]]></given-names>
</name>
<name>
<surname><![CDATA[Hofmann]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Schön]]></surname>
<given-names><![CDATA[MP]]></given-names>
</name>
<name>
<surname><![CDATA[Haenssle]]></surname>
<given-names><![CDATA[HA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Reflectance confocal microscopy and Hailey&#8211; Hailey disease: assessment of response to treatment after CO2 laser ablation]]></article-title>
<source><![CDATA[J Dtsch Dermatol Ges]]></source>
<year>2014</year>
<volume>12</volume>
<numero>12</numero>
<issue>12</issue>
<page-range>1135-7</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[Malan]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Xuejingzi]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
<name>
<surname><![CDATA[Si]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Quan]]></surname>
<given-names><![CDATA[SJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hailey-Hailey disease: the role of azathioprine an immunomodulator]]></article-title>
<source><![CDATA[Pan Afr Med J]]></source>
<year>2019</year>
<volume>35</volume>
<page-range>65</page-range></nlm-citation>
</ref>
<ref id="B17">
<label>17.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Nanda]]></surname>
<given-names><![CDATA[KB]]></given-names>
</name>
<name>
<surname><![CDATA[Saldanha]]></surname>
<given-names><![CDATA[CS]]></given-names>
</name>
<name>
<surname><![CDATA[Jacintha]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Kamath]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hailey-hailey disease responding to thalidomide]]></article-title>
<source><![CDATA[Indian J Dermatol]]></source>
<year>2014</year>
<volume>59</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>190-2</page-range></nlm-citation>
</ref>
<ref id="B18">
<label>18.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Cialfi]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Calabro]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Franchitto]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Zonfrilli]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Screpanti]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Talora]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hypotonic,Acidic Oxidizing Solution Containing Hypochlorous Acid (HClO) as a Potential Treatment of Hailey-Hailey Disease]]></article-title>
<source><![CDATA[Molecules]]></source>
<year>2019</year>
<volume>24</volume>
<numero>24</numero>
<issue>24</issue>
<page-range>4427</page-range></nlm-citation>
</ref>
<ref id="B19">
<label>19.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Sonthalia]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Agrawal]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Talwar]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Goldust]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Low-Dose Naltrexone-Induced Remission in Hailey-Hailey Disease Maintained in Remission with Topical Combination of Ketamine and Diphenhydramine]]></article-title>
<source><![CDATA[Indian Dermatol Online J]]></source>
<year>2019</year>
<volume>10</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>567-70</page-range></nlm-citation>
</ref>
<ref id="B20">
<label>20.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kieffer]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Le Duff]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Chiaverini]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Lacour]]></surname>
<given-names><![CDATA[JP]]></given-names>
</name>
<name>
<surname><![CDATA[Passeron]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Treatment of Severe Hailey-Hailey Disease With Apremilast]]></article-title>
<source><![CDATA[JAMA Dermatol]]></source>
<year>2018</year>
<volume>154</volume>
<numero>12</numero>
<issue>12</issue>
<page-range>1453-6</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
