Case Letter
Pemphigus vulgaris with exclusive manifestation in one of monozygotic twins: could environmental factors be involved?
Marcela Rosa de Almeida Farid, Roberto Bueno-Filho, Eduardo Antônio Donadi, Ana Maria Roselino
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Department of Internal Medicine, Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo, São Paulo, SP, Brazil
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"tieneTextoCompleto" => true "saludo" => "Dear Editor," "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "692" "paginaFinal" => "694" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Marcela Rosa de Almeida Farid, Roberto Bueno-Filho, Eduardo Antônio Donadi, Ana Maria Roselino" "autores" => array:4 [ 0 => array:2 [ "nombre" => "Marcela Rosa de Almeida" "apellidos" => "Farid" ] 1 => array:2 [ "nombre" => "Roberto" "apellidos" => "Bueno-Filho" ] 2 => array:2 [ "nombre" => "Eduardo Antônio" "apellidos" => "Donadi" ] 3 => array:4 [ "nombre" => "Ana Maria" "apellidos" => "Roselino" "email" => array:1 [ 0 => "amfrosel@fmrp.usp.br" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Department of Internal Medicine, Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo, São Paulo, SP, Brazil" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2269 "Ancho" => 2925 "Tamanyo" => 643473 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A), Lesions on the oral mucosa: erosions on the tongue covered by a whitish pseudomembrane, erosions on the labial mucosa, erosions and crusts on the lower lip. (B), Direct immunofluorescence on oral Tzanck smear cytology: fluorescence with anti-IgG on the keratinocytes cell membrane. (C), Histopathology of the oral mucosa (Hematoxylin & eosin, ×40): acantholytic suprabasal bulla. (D), Skin lesions: erythematous plaques on the abdomen and chest with bullae, erosions and hematic crusts.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Pemphigus vulgaris (PV) affects mainly the mucous membranes, through the production of autoantibodies against desmoglein (Dsg) 3. Anti-Dsg1 and anti-Dsg3 autoantibodies are produced in the mucocutaneous form of the disease. The incidence of PV has been increasing in the northeastern region of the state of São Paulo, Brazil, an endemic region for pemphigus foliaceus (PF),<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Susceptible/protective HLA alleles for PV,<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> agricultural activities, and, more recently, salivary proteins from insect bites<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> have been described in association with PV.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Reports of monozygotic twins affected by PV are rare<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>). The present report describes the third case of monozygotic twin sisters, 43-years-old, of which only one developed PV. In September 2018, the twin with PV had multiple oral erosions (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>a). Direct Immunofluorescence (DIF) on the Tzanck smear showed fluorescence with anti-IgG on the keratinocytes cell membrane (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>b); the oral mucosal biopsy demonstrated acantholytic suprabasal bullae (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>c). Multinucleated keratinocytes suggestive of HSV infection were also observed in the Tzanck smear. DIF on the skin biopsy and indirect immunofluorescence (IIF) with normal skin substrate presented fluorescence with anti-IgG on the keratinocytes cell membrane. Prednisone 40 mg/day, dapsone 50 mg/day, and folic acid 5 mg/day were prescribed. After a few days, lesions appeared on the abdomen, anterior chest region, and back (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>d), with the presence of Nikolsky sign. Biopsy of the lesion on the back showed the presence of an acantholytic suprabasal bulla on histopathology, and interkeratinocyte fluorescence for IgG and C3 on DIF. After eight months of treatment, dapsone was replaced by cyclophosphamide at 50 mg/day. In March 2020, the patient suspended the medication due to the coronavirus pandemic. In June 2020, the oral lesions recurred and prednisone was restarted.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">When the patient had exclusive mucosal lesions, the ELISA test resulted in anti-Dsg1 2.0 U/mL and anti-Dsg3 189.1 U/mL (MBL, Japan, cutoff of 20 U/mL for both), while her sister showed negative values ​​on ELISA (1.6 U/mL and 0.7 U/mL, respectively) and IIF.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Both sisters had two HLA susceptibility alleles for PV: <span class="elsevierStyleItalic">HLA-DRB1</span>*08:04 and <span class="elsevierStyleItalic">HLA-DRB1</span>*14:01, while the <span class="elsevierStyleItalic">HLA-DQA1</span> alleles were not associated with PV.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Moreover, they had two other alleles, <span class="elsevierStyleItalic">HLA-DQB1</span>*03:01, and <span class="elsevierStyleItalic">HLA-DQB1</span>*05:01, which have been described in association with resistance and susceptibility to PF, respectively<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>).</p><p id="par0025" class="elsevierStylePara elsevierViewall">Two years later, only one of the twins had PV. Although they had an identical HLA profile, they had not been exposed to similar environmental factors. Both lived in a region prevalent for PV,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> in the northeastern region of the state of São Paulo, Brazil. However, the affected sister had worked in rural activities, on a chicken farm, for 10 years, while her unaffected twin sister reported having always worked in the urban area. Rural workers are more exposed to pesticides, as well as to another possible PV trigger – blood-sucking insect bites.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">When evaluating the HLA profile and environmental factors in PV triggering, one can detect, in advance, those individuals who are most likely to develop the disease. And the question arises whether by modifying the contributing factors, whenever possible, one could prevent the emergence of PV.</p><p id="par0035" class="elsevierStylePara elsevierViewall">In conclusion, the genotype-environmental factor interactions need to be further explored in the pathogenesis of pemphigus, and the present report illustrates this scenario.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Financial support</span><p id="par0040" class="elsevierStylePara elsevierViewall">This work was partially supported by <span class="elsevierStyleGrantSponsor" id="gs0005">FAPESP</span> (<span class="elsevierStyleItalic">Fundação de Amparo à Pesquisa do Estado de São Paulo</span>; <span class="elsevierStyleGrantNumber" refid="gs0005">#2010/51729-2</span>) and <span class="elsevierStyleGrantSponsor" id="gs0010">FAEPA</span> (<span class="elsevierStyleItalic">Fundação de Apoio ao Ensino, Pesquisa e Assistência</span>).</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Authors' contributions</span><p id="par0045" class="elsevierStylePara elsevierViewall">Marcela Rosa de Almeida Farid: Approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; critical review of the literature.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Roberto Bueno-Filho: Approval of the final version of the manuscript; collection, analysis, and interpretation of data; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the manuscript.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Eduardo Antônio Donadi: Approval of the final version of the manuscript; collection, analysis, and interpretation of data; critical review of the manuscript.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Ana Maria Roselino: Approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; effective participation in research orientation; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature; critical review of the manuscript.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">None declared.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:5 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Financial support" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Authors' contributions" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Conflicts of interest" ] 3 => array:2 [ "identificador" => "xack623238" "titulo" => "Acknowledgments" ] 4 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2021-01-09" "fechaAceptado" => "2021-04-06" "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0020">Study conducted at the Department of Internal Medicine, Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo, São Paulo, SP, Brazil.</p>" ] ] "multimedia" => array:2 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2269 "Ancho" => 2925 "Tamanyo" => 643473 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A), Lesions on the oral mucosa: erosions on the tongue covered by a whitish pseudomembrane, erosions on the labial mucosa, erosions and crusts on the lower lip. (B), Direct immunofluorescence on oral Tzanck smear cytology: fluorescence with anti-IgG on the keratinocytes cell membrane. (C), Histopathology of the oral mucosa (Hematoxylin & eosin, ×40): acantholytic suprabasal bulla. (D), Skin lesions: erythematous plaques on the abdomen and chest with bullae, erosions and hematic crusts.</p>" ] ] 1 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:3 [ "leyenda" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">IIF, Indirect immunofluorescence; Dsg, Desmoglein; ND, Not determined; NA, Not affected.</p>" "tablatextoimagen" => array:1 [ 0 => array:1 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Reference \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Clinical form of pemphigus vulgaris \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Sex \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Age (years) \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">IIF \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Anti-Dsg1 (U/mL) \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Anti-Dsg3 (U/mL) \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">HLA Alleles/haplotypes<a class="elsevierStyleCrossRef" href="#tblfn0015"><span class="elsevierStyleSup">c</span></a> \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Ruocco et al. (1985)<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Italy</td><td class="td" title="\n \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">Twin 1 \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 \t\t\t\t\ttop\n \t\t\t\t">Mucocutaneous \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 " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Female</td><td class="td" title="\n \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">11½ \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 \t\t\t\t\ttop\n \t\t\t\t">1:40 \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 \t\t\t\t\ttop\n \t\t\t\t">ND \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 \t\t\t\t\ttop\n \t\t\t\t">ND \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 " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">A</span>*24, <span class="elsevierStyleItalic">A</span>*29; <span class="elsevierStyleItalic">B</span>*45, B*35; <span class="elsevierStyleItalic">C</span>*04, C*x; <span class="elsevierStyleItalic">DRB1</span>*04, <span class="elsevierStyleItalic">DRB1</span>*07</td></tr><tr title="table-row"><td class="td" title="\n \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">Twin 2 \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 \t\t\t\t\ttop\n \t\t\t\t">Not affected<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> \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 \t\t\t\t\ttop\n \t\t\t\t">NA \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 \t\t\t\t\ttop\n \t\t\t\t">1:20 \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 \t\t\t\t\ttop\n \t\t\t\t">ND \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 \t\t\t\t\ttop\n \t\t\t\t">ND \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Salathiel et al. (2016)<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> \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 \t\t\t\t\ttop\n \t\t\t\t">Twin 1 \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 " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Mucocutaneous</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Female</td><td class="td" title="\n \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">11 \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 \t\t\t\t\ttop\n \t\t\t\t">IgG \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 \t\t\t\t\ttop\n \t\t\t\t">120.77 \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 \t\t\t\t\ttop\n \t\t\t\t">160.82 \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 \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic"><span class="elsevierStyleUnderline">DRB1</span></span><span class="elsevierStyleUnderline">*04:02</span>-<span class="elsevierStyleUnderline">D</span><span class="elsevierStyleItalic"><span class="elsevierStyleUnderline">QA1</span></span><span class="elsevierStyleUnderline">*03:01</span>-<span class="elsevierStyleItalic"><span class="elsevierStyleUnderline">DQB1</span></span><span class="elsevierStyleUnderline">*03:02</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Brazil \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 \t\t\t\t\ttop\n \t\t\t\t">Twin 2 \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 \t\t\t\t\ttop\n \t\t\t\t">16 \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 \t\t\t\t\ttop\n \t\t\t\t">IgG \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 \t\t\t\t\ttop\n \t\t\t\t">74.02 \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 \t\t\t\t\ttop\n \t\t\t\t">151.66 \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 \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic"><span class="elsevierStyleUnderline">DRB1</span></span><span class="elsevierStyleUnderline">*14:04</span>-<span class="elsevierStyleItalic"><span class="elsevierStyleUnderline">DQA1</span></span><span class="elsevierStyleUnderline">*01:01</span>-<span class="elsevierStyleItalic"><span class="elsevierStyleUnderline">DQB1</span></span><span class="elsevierStyleUnderline">*05:03</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Farid et al. (2021) \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 \t\t\t\t\ttop\n \t\t\t\t">Twin 1 \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 \t\t\t\t\ttop\n \t\t\t\t">Mucocutaneous \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 " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Female</td><td class="td" title="\n \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">43 \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 \t\t\t\t\ttop\n \t\t\t\t">IgG \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 \t\t\t\t\ttop\n \t\t\t\t">2.0± \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 \t\t\t\t\ttop\n \t\t\t\t">189.1 \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 " rowspan="2" align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic"><span class="elsevierStyleUnderline">DRB1</span></span><span class="elsevierStyleUnderline">*08:04</span>, <span class="elsevierStyleItalic"><span class="elsevierStyleUnderline">DRB1</span></span><span class="elsevierStyleUnderline">*14:01</span>; <span class="elsevierStyleItalic">DQA1</span>*01:01, <span class="elsevierStyleItalic">DQA1</span>*05:01; <span class="elsevierStyleItalic">DQB1</span>*03:01<span class="elsevierStyleSup">+</span>, <span class="elsevierStyleItalic">DQB1</span>*05:01<span class="elsevierStyleSup">++</span></td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Brazil (current) \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 \t\t\t\t\ttop\n \t\t\t\t">Twin 2 \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 \t\t\t\t\ttop\n \t\t\t\t">Not affected<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">b</span></a> \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 \t\t\t\t\ttop\n \t\t\t\t">NA \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 \t\t\t\t\ttop\n \t\t\t\t">IgG \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 \t\t\t\t\ttop\n \t\t\t\t">1.6 \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 \t\t\t\t\ttop\n \t\t\t\t">0.7 \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] ] ] "notaPie" => array:3 [ 0 => array:3 [ "identificador" => "tblfn0005" "etiqueta" => "a" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">At 23 years old.</p>" ] 1 => array:3 [ "identificador" => "tblfn0010" "etiqueta" => "b" "nota" => "<p class="elsevierStyleNotepara" id="npar0010">At 45 years old; ± anti-Dsg1 titer when the patient had exclusive oral lesions.</p>" ] 2 => array:3 [ "identificador" => "tblfn0015" "etiqueta" => "c" "nota" => "<p class="elsevierStyleNotepara" id="npar0015">PV susceptibility alleles are underlined; resistance + and susceptibility++ alleles to pemphigus foliaceus (see Ref. <a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a>.</p>" ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Clinical and laboratory data obtained from the literature on monozygotic twins presenting with pemphigus vulgaris.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Geographical foci and epidemiological changes of pemphigus vulgaris in four decades in Southeastern Brazil" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "B.S. Celere" 1 => "S. Vernal" 2 => "M.J.F. Brochado" 3 => "S.I. Segura-Muñoz" 4 => "A.M. Roselino" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/ijd.13714" "Revista" => array:6 [ "tituloSerie" => "Int J Dermatol." "fecha" => "2017" "volumen" => "56" "paginaInicial" => "1494" "paginaFinal" => "1496" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28737218" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Differential HLA class I and class II associations in pemphigus foliaceus and pemphigus vulgaris patients from a prevalent Southeastern Brazilian region" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "M.J. Brochado" 1 => "D.F. Nascimento" 2 => "W. Campos" 3 => "N.H.S. Deghaide" 4 => "E.A. Donadi" 5 => "A.M. Roselino" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jaut.2016.04.007" "Revista" => array:6 [ "tituloSerie" => "J Autoimmun." "fecha" => "2016" "volumen" => "72" "paginaInicial" => "19" "paginaFinal" => "24" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27178774" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Image Gallery: A case of pemphigus vulgaris following Simulium spp. (Diptera) bites" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "C.G. Wambier" 1 => "T.A. Struecker" 2 => "L.N. Durski" 3 => "A.G. Araújo" 4 => "S.P.F. Wambier" 5 => "M.A. Cappel" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/bjd.15393" "Revista" => array:5 [ "tituloSerie" => "Br J Dermatol." "fecha" => "2017" "volumen" => "176" "paginaInicial" => "e100" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28504401" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pemphigus vulgaris in only one of two monozygotic twins" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "V. Ruocco" 1 => "G. Peluso" 2 => "M. Pisani" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/s0190-9622(85)80104-3" "Revista" => array:6 [ "tituloSerie" => "J Am Acad Dermatol." "fecha" => "1985" "volumen" => "12" "paginaInicial" => "587" "paginaFinal" => "589" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/4039336" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Family study of monozygotic twins affected by pemphigus vulgaris" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "A.M. Salathiel" 1 => "M.J. Brochado" 2 => "O. Kim" 3 => "N.H.S. Deghaide" 4 => "E.A. Donadi" 5 => "A.M. Roselino" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.humimm.2016.05.005" "Revista" => array:6 [ "tituloSerie" => "Hum Immunol." "fecha" => "2016" "volumen" => "77" "paginaInicial" => "600" "paginaFinal" => "604" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27177496" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] "agradecimientos" => array:1 [ 0 => array:4 [ "identificador" => "xack623238" "titulo" => "Acknowledgments" "texto" => "<p id="par0070" class="elsevierStylePara elsevierViewall">The authors would like to thank Dr. Leticia Arruda Camargo and Tamiris Amanda Julio, PhD, for their contribution in the preparation of the clinical case and in the assessment of anti-desmogleins by ELISA, respectively.</p>" "vista" => "all" ] ] ] "idiomaDefecto" => "en" "url" => "/03650596/0000009700000005/v1_202208290604/S0365059622001222/v1_202208290604/en/main.assets" "Apartado" => array:4 [ "identificador" => "82769" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Case Letter" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/03650596/0000009700000005/v1_202208290604/S0365059622001222/v1_202208290604/en/main.pdf?idApp=UINPBA00008Z&text.app=https://clinics.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0365059622001222?idApp=UINPBA00008Z" ]
| Ano/Mês | Html | Total | |
|---|---|---|---|
| 2024 Novembro | 18 | 7 | 25 |
| 2024 Outubro | 102 | 64 | 166 |
| 2024 Setembro | 124 | 94 | 218 |
| 2024 Agosto | 134 | 113 | 247 |
| 2024 Julho | 122 | 103 | 225 |
| 2024 Junho | 88 | 65 | 153 |
| 2024 Maio | 82 | 70 | 152 |
| 2024 Abril | 98 | 95 | 193 |
| 2024 Março | 79 | 60 | 139 |
| 2024 Fevereiro | 86 | 80 | 166 |
| 2024 Janeiro | 95 | 58 | 153 |
| 2023 Dezembro | 76 | 57 | 133 |
| 2023 Novembro | 57 | 67 | 124 |
| 2023 Outubro | 58 | 73 | 131 |
| 2023 Setembro | 69 | 85 | 154 |
| 2023 Agosto | 36 | 31 | 67 |
| 2023 Julho | 62 | 27 | 89 |
| 2023 Junho | 61 | 41 | 102 |
| 2023 Maio | 37 | 19 | 56 |
| 2023 Abril | 27 | 16 | 43 |
| 2023 Março | 50 | 50 | 100 |
| 2023 Fevereiro | 40 | 34 | 74 |
| 2023 Janeiro | 42 | 47 | 89 |
| 2022 Dezembro | 75 | 43 | 118 |
| 2022 Novembro | 82 | 63 | 145 |
| 2022 Outubro | 115 | 70 | 185 |
| 2022 Setembro | 114 | 104 | 218 |
| 2022 Agosto | 62 | 43 | 105 |
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