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Descripción de un caso" "tienePdf" => "es" "tieneTextoCompleto" => "es" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "526" "paginaFinal" => "528" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Pemphigus Herpetiformis With Progression To Pemphigus Foliaceus: A Case Report" ] ] "contieneTextoCompleto" => array:1 [ "es" => true ] "contienePdf" => array:1 [ "es" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figura 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 831 "Ancho" => 1429 "Tamanyo" => 208685 ] ] "descripcion" => array:1 [ "es" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A. Placa eritematosa descamativa localizada en la zona retroauricular. B. 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Fuentes-Finkelstein, M. Barnadas, C. Gelpi, L. Puig" "autores" => array:4 [ 0 => array:4 [ "nombre" => "P." "apellidos" => "Fuentes-Finkelstein" "email" => array:1 [ 0 => "pfuentesf@santpau.cat" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "M." "apellidos" => "Barnadas" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "C." "apellidos" => "Gelpi" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "L." "apellidos" => "Puig" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio Dermatología, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Inmunología, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Pénfigo herpetiforme con evolución a pénfigo foliáceo. Descripción de un caso" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 752 "Ancho" => 1293 "Tamanyo" => 188891 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A, Erythematous scaling plaque in the retroauricular area. B, Acantholytic subcorneal vesicle (hematoxylin eosin, original magnification ×400).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Pemphigus herpetiformis (PH) is an uncommon variant of pemphigus that accounts for an estimated 6% to 7.2% of all cases of this skin disorder.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> The term <span class="elsevierStyleItalic">pemphigus herpetiformis</span> was coined by Jablonska et al.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> in 1975 to describe a entity that was clinically similar to dermatitis herpetiformis, showed acantholysis on biopsy, and responded to sulfapyridine. The authors considered that PH was a variant of pemphigus based on the direct immunofluorescence findings. PH typically presents with annular erythematous plaques, peripheral vesicles, and on occasions intense pruritus. Histologic features are highly variable and depend on the stage of the lesion. There have been reports of PH preceding or developing concurrently with pemphigus foliaceous (PF) or pemphigus vulgaris (PV).<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3–5</span></a> We present a case of PH that progressed to PF.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The patient, a 34-year-old woman with no relevant past history, consulted for erythematous papules and plaques with peripheral vesicles and blisters on the lower extremities, the abdomen, and the scalp (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The lesions had been present for 3 months. There was no mucosal involvement. Two biopsies revealed different degrees of neutrophilic and eosinophilic spongiosis (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>A) and acantholysis, with the formation of intraepidermal vesicles (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>B). Several eosinophils were also observed in the papillary dermis. Direct immunofluorescence showed intercellular immunoglobulin (Ig) G and C3 deposits, predominantly in the suprabasal layers of the epidermis. Anti-epithelial antibodies (titer, 1:40) and anti-desmoglein 1 (Dsg1) antibodies were positive (175 IU/mL; normal value, <<span class="elsevierStyleHsp" style=""></span>20 IU/mL); the results for anti-Dsg1 antibodies were negative. A diagnosis of PH was made and treatment was started with prednisone 20<span class="elsevierStyleHsp" style=""></span>mg/d and topical clobetasol. The lesions improved, but 3 months later, erythematous scaling plaques started to reappear in the presternal, dorsal, and retroauricular areas and on the scalp (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>A). Biopsy of one of these plaques showed a subcorneal acantholytic vesicle (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>B). The anti-epithelial antibody titer was 1:80 and anti-Dsg1 antibody levels remained high at 161<span class="elsevierStyleHsp" style=""></span>IU/mL. Given the persistence of the lesions, treatment was started with dapsone 50<span class="elsevierStyleHsp" style=""></span>mg/d, with dose increments up to 100<span class="elsevierStyleHsp" style=""></span>mg/d. The clinical response was favorable and the dose was progressively reduced to 12.5<span class="elsevierStyleHsp" style=""></span>mg every second day. Occasional flares consisting of minimal papules with scaling on the neckline, back, and scalp were observed.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">PH is a variant of pemphigus that generally has a good prognosis, and most patients respond to sulfones.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> PH has a wide spectrum of clinical and histologic findings and accordingly numerous autoimmune blistering disorders must be considered in the differential diagnosis, namely, dermatitis herpetiformis, linear IgA bullous dermatosis, PF, PV, and bullous pemphigoid.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> Histologic findings can vary according to the stage of the disease and the type of lesion biopsied. Varying degrees of neutrophilic and/or eosinophilic spongiosis, with or without acantholysis in the middle and/or subcorneal layer, are observed. Kuhn et al.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> found that the inflammatory infiltrate in patients with PH was 68% eosinophil-dominant, 16% neutrophil-dominant, and 16% mixed eosinophil/neutrophil. We would like to stress the importance of performing direct immunofluorescence to test for the presence of an autoimmune blistering disorder when histology reveals neutrophilic and/or eosinophilic spongiosis.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Anti-Dsg1 and/or anti-Dsg3 antibodies have been described in most cases of pH,<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5,8</span></a> but there have been isolated reports of negative results for both antibodies.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">We have described an atypical course of PH that has been previously reported by Maciejowska et al.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> in 2 of 15 patients and by Santi et al.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> in 1 of 7 patients. There have also been reports of cases of PH developing after or at the same time as PF or PV.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,5</span></a> This possibility, together with the fact that anti-Dsg1 antibodies are detected in PH, has led to the hypothesis that PH and PF might be connected and that PH might actually be a variant of PF.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">It is not known why patients with PH, despite having anti-Dsg1 antibodies, have different clinical manifestations and histological findings to those with PF. Several hypotheses have been proposed. One is that in PH, IgG might cause keratinocytes to induce interleukin 8, whose chemotactic activity would explain the presence of a neutrophilic infiltrate.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> Another is that patients might develop antibodies that, despite their minimum acantholytic activity, could activate eosinophils and neutrophils through the Fc portion of IgG.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Finally, it is possible that antibodies targeting epidermal antigens other than desmogleins or different epitopes might be responsible for the different phenotypic expression of pemphigus seen in PH.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Fuentes-Finkelstein P, Barnadas M, Gelpi C, Puig L. Pénfigo herpetiforme con evolución a pénfigo foliáceo. Descripción de un caso. Actas Dermosifiliogr. 2014;105:533–535.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1004 "Ancho" => 754 "Tamanyo" => 106826 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Erythematous papules and plaques with circinate borders and peripheral vesicles.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 867 "Ancho" => 1301 "Tamanyo" => 276426 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Neutrophilic spongiosis in the epidermis with isolated eosinophils (hematoxylin eosin, original magnification ×400). B, Acantholytic vesicle in the middle layer of the epidermis accompanied by neutrophils and eosinophils.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 752 "Ancho" => 1293 "Tamanyo" => 188891 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A, Erythematous scaling plaque in the retroauricular area. B, Acantholytic subcorneal vesicle (hematoxylin eosin, original magnification ×400).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Is pemphigus herpetiformis an entity?" 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año/Mes | Html | Total | |
---|---|---|---|
2024 Noviembre | 12 | 15 | 27 |
2024 Octubre | 139 | 59 | 198 |
2024 Septiembre | 151 | 28 | 179 |
2024 Agosto | 162 | 73 | 235 |
2024 Julio | 192 | 40 | 232 |
2024 Junio | 116 | 38 | 154 |
2024 Mayo | 130 | 37 | 167 |
2024 Abril | 100 | 31 | 131 |
2024 Marzo | 122 | 32 | 154 |
2024 Febrero | 102 | 39 | 141 |
2024 Enero | 98 | 35 | 133 |
2023 Diciembre | 105 | 31 | 136 |
2023 Noviembre | 106 | 33 | 139 |
2023 Octubre | 84 | 46 | 130 |
2023 Septiembre | 109 | 42 | 151 |
2023 Agosto | 70 | 20 | 90 |
2023 Julio | 94 | 36 | 130 |
2023 Junio | 83 | 28 | 111 |
2023 Mayo | 117 | 30 | 147 |
2023 Abril | 82 | 23 | 105 |
2023 Marzo | 69 | 26 | 95 |
2023 Febrero | 77 | 19 | 96 |
2023 Enero | 64 | 20 | 84 |
2022 Diciembre | 75 | 42 | 117 |
2022 Noviembre | 60 | 27 | 87 |
2022 Octubre | 41 | 24 | 65 |
2022 Septiembre | 44 | 49 | 93 |
2022 Agosto | 70 | 33 | 103 |
2022 Julio | 34 | 27 | 61 |
2022 Junio | 55 | 30 | 85 |
2022 Mayo | 97 | 43 | 140 |
2022 Abril | 100 | 35 | 135 |
2022 Marzo | 117 | 72 | 189 |
2022 Febrero | 92 | 24 | 116 |
2022 Enero | 74 | 40 | 114 |
2021 Diciembre | 58 | 42 | 100 |
2021 Noviembre | 105 | 53 | 158 |
2021 Octubre | 95 | 42 | 137 |
2021 Septiembre | 115 | 30 | 145 |
2021 Agosto | 145 | 40 | 185 |
2021 Julio | 147 | 31 | 178 |
2021 Junio | 84 | 24 | 108 |
2021 Mayo | 80 | 25 | 105 |
2021 Abril | 205 | 104 | 309 |
2021 Marzo | 111 | 35 | 146 |
2021 Febrero | 69 | 27 | 96 |
2021 Enero | 33 | 19 | 52 |
2020 Diciembre | 53 | 12 | 65 |
2020 Noviembre | 47 | 6 | 53 |
2020 Octubre | 62 | 9 | 71 |
2020 Septiembre | 80 | 10 | 90 |
2020 Agosto | 63 | 19 | 82 |
2020 Julio | 46 | 25 | 71 |
2020 Junio | 44 | 21 | 65 |
2020 Mayo | 32 | 16 | 48 |
2020 Abril | 29 | 15 | 44 |
2020 Marzo | 31 | 12 | 43 |
2020 Febrero | 3 | 3 | 6 |
2020 Enero | 0 | 5 | 5 |
2019 Diciembre | 4 | 5 | 9 |
2019 Noviembre | 0 | 2 | 2 |
2019 Octubre | 0 | 3 | 3 |
2019 Septiembre | 4 | 5 | 9 |
2019 Agosto | 2 | 2 | 4 |
2019 Julio | 0 | 4 | 4 |
2019 Junio | 3 | 7 | 10 |
2019 Mayo | 1 | 29 | 30 |
2019 Abril | 14 | 1 | 15 |
2019 Marzo | 2 | 8 | 10 |
2019 Febrero | 1 | 0 | 1 |
2019 Enero | 2 | 1 | 3 |
2018 Diciembre | 1 | 0 | 1 |
2018 Noviembre | 2 | 0 | 2 |
2018 Octubre | 3 | 0 | 3 |
2018 Septiembre | 5 | 0 | 5 |
2018 Abril | 0 | 1 | 1 |
2018 Marzo | 3 | 4 | 7 |
2018 Febrero | 30 | 2 | 32 |
2018 Enero | 48 | 5 | 53 |
2017 Diciembre | 40 | 5 | 45 |
2017 Noviembre | 32 | 4 | 36 |
2017 Octubre | 37 | 5 | 42 |
2017 Septiembre | 42 | 12 | 54 |
2017 Agosto | 58 | 6 | 64 |
2017 Julio | 63 | 10 | 73 |
2017 Junio | 67 | 44 | 111 |
2017 Mayo | 50 | 15 | 65 |
2017 Abril | 53 | 13 | 66 |
2017 Marzo | 43 | 29 | 72 |
2017 Febrero | 39 | 5 | 44 |
2017 Enero | 25 | 6 | 31 |
2016 Diciembre | 41 | 13 | 54 |
2016 Noviembre | 55 | 13 | 68 |
2016 Octubre | 83 | 7 | 90 |
2016 Septiembre | 108 | 9 | 117 |
2016 Agosto | 46 | 2 | 48 |
2016 Julio | 41 | 8 | 49 |
2016 Junio | 5 | 1 | 6 |
2016 Mayo | 2 | 4 | 6 |
2016 Abril | 4 | 8 | 12 |
2016 Marzo | 5 | 7 | 12 |
2016 Febrero | 4 | 11 | 15 |
2016 Enero | 14 | 1 | 15 |
2015 Diciembre | 9 | 5 | 14 |
2015 Noviembre | 3 | 5 | 8 |
2015 Octubre | 4 | 1 | 5 |
2015 Septiembre | 2 | 5 | 7 |
2015 Agosto | 5 | 3 | 8 |
2015 Julio | 42 | 8 | 50 |
2015 Junio | 35 | 6 | 41 |
2015 Mayo | 46 | 12 | 58 |
2015 Abril | 40 | 8 | 48 |
2015 Marzo | 39 | 8 | 47 |
2015 Febrero | 42 | 7 | 49 |
2015 Enero | 29 | 5 | 34 |
2014 Diciembre | 45 | 5 | 50 |
2014 Noviembre | 40 | 9 | 49 |
2014 Octubre | 27 | 3 | 30 |
2014 Septiembre | 16 | 4 | 20 |
2014 Agosto | 15 | 4 | 19 |
2014 Julio | 9 | 7 | 16 |
2014 Junio | 6 | 5 | 11 |
2014 Mayo | 2 | 1 | 3 |