Letter - Therapy
Successful treatment of erythrodermic pemphigus foliaceus with intravenous immunoglobulin
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(B) Complete resolution with therapy</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Pemphigus foliaceus (PF) is characterized by the presence of superficial vesicles or bullae in the absence of mucosal involvement, it results from the interaction of IgG autoantibodies with desmoglein 1, present in the upper layers of the epidermis.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> PF is endemically present in Brazil and other South American countries<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> and can manifest as localized and disseminated forms. The disseminated forms are subdivided into four clinical variants, vesico-bullous, keratotic, herpetiform and erythrodermic. In the latter, the entire tegument is erythematous and desquamative, with areas of erosion, exudation, and crusts.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">This case report describes a 68-year-old female patient, who started showing erythematous-desquamative lesions on the face, upper trunk and arms, with circinate edges and lamellar desquamation (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A). The edge of one lesion was biopsied and an upper intraepidermal cleavage was demonstrated on histopathology, along with acantholytic cells (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>), confirming the diagnosis of classic PF, since the patient does not come from an endemic area. Additional confirmation of the diagnosis was obtained through immunofluorescence, which showed an intercellular epidermal pattern of IgG deposition.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Therapy with 60 mg of oral prednisone was implemented but there was acral expansion of the condition in the following six months, leading to lamellar desquamation of the plantar regions (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B). Oral methotrexate (15 mg per week) was added to the therapy, which the patient did not tolerate. The condition continued to expand until it became erythrodermic after six months (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>A). Therapy was then instituted with intravenous (IV) immunoglobulin, at a total dose of 2 g/Kg/cycle, infused on five consecutive days; a total of four cycles were applied with four-week intervals between one and the next. Relevant clinical improvement was observed (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>B), with no side effects, which allowed a significant reduction in the oral corticosteroid use, which is currently at 5 mg daily, after a favorable 14-month follow-up.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The use of IV immunoglobulin is well established in autoimmune diseases, including pemphigus,<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,4</span></a> and is recommended for refractory cases such as this one.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Its action mechanism is probably multiple, with the most relevant being perhaps receptor saturation, with consequent immune cell inhibition. In view of the COVID-19 pandemic, the use of rituximab as a therapeutic alternative has been questioned, due to the intense inhibition of the humoral immune response, and so IV immunoglobulin is an excellent option.</p><p id="par0025" class="elsevierStylePara elsevierViewall">There is only one Brazilian report of an adolescent patient with endemic pemphigus foliaceus,<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> who also developed the erythrodermic form and was treated with IV immunoglobulin. The case reported herein documents the successful use of this therapy in severe and extensive cases of pemphigus foliaceus, which is rare in this age group.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Financial support</span><p id="par0030" class="elsevierStylePara elsevierViewall">None declared.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Authors' contributions</span><p id="par0035" class="elsevierStylePara elsevierViewall">Hiram Almeida Jr: 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; critical review of the manuscript.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Junior Wieczorek: 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 manuscript.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Mahony Santana: 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 manuscript.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Celina Leite: 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 manuscript;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">None declared.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:4 [ 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:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2022-03-09" "fechaAceptado" => "2022-05-24" "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Study conducted at the Universidade Católica de Pelotas, Pelotas, RS, Brazil.</p>" ] ] "multimedia" => array:3 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1888 "Ancho" => 2175 "Tamanyo" => 380070 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Erythematous-desquamative lesions on the arm, with circinate edges and lamellar desquamation. (B) Plantar lamellar desquamation</p>" ] ] 1 => array:8 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1454 "Ancho" => 1675 "Tamanyo" => 329195 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Histopathology showing intraepidermal cleavage and acantholytic keratinocytes (arrows); Hematoxylin & eosin, ×200</p>" ] ] 2 => array:8 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1708 "Ancho" => 2175 "Tamanyo" => 312749 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0015" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">(A) Desquamation of the entire face before treatment. (B) Complete resolution with therapy</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" => "Update on fogo selvagem, an endemic form of pemphigus foliaceus" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "V. Aoki" 1 => "E.A. Rivitti" 2 => "L.A. 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| Ano/Mês | Html | Total | |
|---|---|---|---|
| 2024 Novembro | 28 | 4 | 32 |
| 2024 Outubro | 169 | 68 | 237 |
| 2024 Setembro | 190 | 92 | 282 |
| 2024 Agosto | 231 | 119 | 350 |
| 2024 Julho | 182 | 111 | 293 |
| 2024 Junho | 134 | 65 | 199 |
| 2024 Maio | 122 | 59 | 181 |
| 2024 Abril | 166 | 99 | 265 |
| 2024 Março | 203 | 81 | 284 |
| 2024 Fevereiro | 552 | 99 | 651 |
| 2024 Janeiro | 397 | 88 | 485 |
| 2023 Dezembro | 54 | 53 | 107 |
| 2023 Novembro | 94 | 89 | 183 |
| 2023 Outubro | 48 | 70 | 118 |
| 2023 Setembro | 19 | 19 | 38 |
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