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"apellidos" => "Monteagudo" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0001731017302673" "doi" => "10.1016/j.ad.2017.04.019" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0001731017302673?idApp=UINPBA000044" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219017302627?idApp=UINPBA000044" "url" => "/15782190/0000010800000009/v1_201711021004/S1578219017302627/v1_201711021004/en/main.assets" ] "en" => array:16 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Case and Research Letter</span>" "titulo" => "Linear IgA Bullous Dermatosis Presenting as Toxic Epidermal Necrolysis" "tieneTextoCompleto" => true "saludo" => "<span class="elsevierStyleItalic">To the Editor:</span>" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "880" "paginaFinal" => "882" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "J. Ruiz-Rivero, I. Hernández-Aragüés, A. Pulido-Pérez, R. Suárez-Fernández" "autores" => array:4 [ 0 => array:4 [ "nombre" => "J." "apellidos" => "Ruiz-Rivero" "email" => array:1 [ 0 => "juncalrr@gmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "I." "apellidos" => "Hernández-Aragüés" ] 2 => array:2 [ "nombre" => "A." "apellidos" => "Pulido-Pérez" ] 3 => array:2 [ "nombre" => "R." "apellidos" => "Suárez-Fernández" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Dermatología y Venereología Médico-Quirúrgica, Hospital General Universitario Gregorio Marañón, Madrid, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Dermatosis ampollosa IgA lineal con presentación clínica de necrólisis epidérmica tóxica" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 861 "Ancho" => 2499 "Tamanyo" => 279122 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Onset of the lesions as large desquamating erythematous plaques. B, Subsequent formation of erosions.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Linear immunoglobulin (Ig) A dermatosis (LAD) is a rare autoimmune bullous disease characterized by the formation of subepidermal blisters with linear deposits of IgA along the basement membrane, visible on direct immunofluorescence.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1–3</span></a> Cases of LAD mimicking other blistering diseases, such as bullous pemphigoid, pemphigus vulgaris, dermatitis herpetiformis, and impetigo, and nonbullous diseases have been reported.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,5</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of an 88-year-old woman receiving palliative care for metastatic squamous cell carcinoma of the vulva. She was admitted to internal medicine for cellulitis of the right lower limb, for which the portal of entry was an ulcer in the right inguinal region. Computed tomography revealed a right inguinal fluid collection of 11<span class="elsevierStyleHsp" style=""></span>cm in diameter, with gas, and osteomyelitis of the pubis that required urgent surgical debridement. The patient developed sepsis and treatment was started with ertapenem and vancomycin. Five days later, the dermatology department was consulted for the appearance of widespread desquamating erythematous lesions on the face, trunk, and limbs (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A). Despite withdrawal of the vancomycin for suspected toxic epidermal necrolysis (TEN), LAD, or other drug-related hypersensitivity syndromes, the condition progressed to erythroderma in the space of 24<span class="elsevierStyleHsp" style=""></span>hours, with tense blisters, erosions, and epidermal separation in pressure areas (<a class="elsevierStyleCrossRef" href="#fig0005">Figs. 1</a>B and 2A), with involvement of the oral mucosa, but not of other mucosas. In view of the patient's basal situation, she was not a candidate for transfer to a specialist burns unit. Treatment was started with systemic corticosteroids and, given the lack of response, it was decided to administer immunoglobulins at a dose of 0.4<span class="elsevierStyleHsp" style=""></span>g/kg/d for 5 days. However, the condition progressed to widespread epidermal detachment (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>B) and the patient died 7 days later. Skin biopsy (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>A) revealed an epidermis with a normal maturation gradient and subepidermal vesicles full of a fibrinoid material with polymorphonuclear cells and eosinophils; these findings were consistent with a diagnosis of LAD. The superficial dermis showed a mild perivascular lymphocytic and eosinophilic inflammatory infiltrate. On direct immunofluorescence (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>B), a linear deposit of IgA was visible along the dermoepidermal junction, with no other pathological deposits with the antisera tested (fibrinogen, IgG, IgM, and complement component C3). Indirect immunofluorescence on 1<span class="elsevierStyleHsp" style=""></span>M sodium chloride-separated skin, showed the deposits to be on the epidermal side of the vesicles.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">The first descriptions of LAD are attributed to Bowen in 1901, although it was not until 1979 when it was considered a separate entity from dermatitis herpetiformis.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> LAD is a clinically and histologically heterogeneous condition that includes disorders with distinct clinical courses and whose diagnostic criteria continue under discussion.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> There are 2 main forms. The infantile form of LAD is characterized by erythematous plaques with tense peripheral blisters with the <span class="elsevierStyleItalic">string of pearls</span> sign; this form preferentially affects the trunk, perineum, and perioral region. Although the clinical course is variable, infantile LAD tends to resolve spontaneously before puberty.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,3</span></a> The adult form is much more variable and is sometimes considered to mimic other diseases.</p><p id="par0020" class="elsevierStylePara elsevierViewall">A number of antigens have been reported to be implicated in the etiology of LAD, most importantly BP180, BP230, LAD1, and LAD97 localized on the epidermal side on indirect immunofluorescence of separated skin, and collagen VII, localized on the dermal side<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,4,7</span></a>; it is this diversity of antigens that is thought to explain the clinical heterogeneity of the disease.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> Drug-induced and idiopathic forms are traditionally recognized. The drug-related form, occurring most commonly with vancomycin, followed by phenytoin,<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,8,9</span></a> develops between 1 and 30 days after starting treatment. The possibility of a tumor-related form (particularly with hematologic tumors) has also been suggested. The form similar to TEN is perhaps the most striking mode of clinical presentation of LAD, as it is most commonly associated with erythroderma, a positive Nikolsky sign, and palmoplantar and mucosal involvement; vancomycin also the most frequently implicated drug.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">The treatment of choice is dapsone, though corticosteroids, azathioprine, methotrexate, ciclosporin, and immunoglobulins are occasionally used.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,3,10</span></a> The treatment of LAD that presents as TEN is no different from that of other forms of LAD, although the greater extension and severity of the skin lesions requires greater attention to physical measures. Withdrawal of the suspected drug, when there is one, is the first and sometimes only step. On other occasions it is necessary to resort to systemic corticosteroids. The prognosis will also depend on the patient's basal situation and comorbid conditions.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,3,7</span></a> The use of immunoglobulins in LAD, as in other blistering diseases,<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> is reserved for severe and refractory cases.</p><p id="par0030" class="elsevierStylePara elsevierViewall">In summary, LAD is a heterogeneous entity that, among its numerous forms of presentation, can mimic TEN.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,5,7,9</span></a> It is important to include LAD in the differential diagnosis of vesiculobullous dermatoses and of TEN, making it advisable routinely to perform not only histological study in these cases but also immunofluorescence.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of Interest</span><p id="par0035" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of Interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Ruiz-Rivero J, Hernández-Aragüés I, Pulido-Pérez A, Suárez-Fernández R. Dermatosis ampollosa IgA lineal con presentación clínica de necrólisis epidérmica tóxica. Actas Dermosifiliogr. 2017;108:880–882.</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" => 861 "Ancho" => 2499 "Tamanyo" => 279122 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Onset of the lesions as large desquamating erythematous plaques. B, Subsequent formation of erosions.</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" => 936 "Ancho" => 2501 "Tamanyo" => 289811 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Formation of tense blisters on the thigh. B, Final stage of widespread epidermal detachment.</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" => 1281 "Ancho" => 2499 "Tamanyo" => 543623 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A, Histology. A subepidermal blister is visible, containing fibrinoid elements and polymorphonuclear cells and eosinophils. Hematoxylin and eosin, original magnification ×10. B, Direct immunofluorescence. Linear deposits of immunoglobulin A are visible along the dermoepidermal junction.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Linear IgA bullous dermatosis: A retrospective study of 23 patients in Denmark" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "K. Lings" 1 => "A. 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Year/Month | Html | Total | |
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2024 November | 11 | 8 | 19 |
2024 October | 68 | 34 | 102 |
2024 September | 79 | 36 | 115 |
2024 August | 126 | 64 | 190 |
2024 July | 85 | 26 | 111 |
2024 June | 110 | 41 | 151 |
2024 May | 76 | 26 | 102 |
2024 April | 89 | 32 | 121 |
2024 March | 88 | 33 | 121 |
2024 February | 66 | 27 | 93 |
2024 January | 68 | 37 | 105 |
2023 December | 52 | 20 | 72 |
2023 November | 64 | 21 | 85 |
2023 October | 65 | 17 | 82 |
2023 September | 68 | 34 | 102 |
2023 August | 49 | 20 | 69 |
2023 July | 57 | 25 | 82 |
2023 June | 56 | 24 | 80 |
2023 May | 73 | 18 | 91 |
2023 April | 54 | 38 | 92 |
2023 March | 64 | 32 | 96 |
2023 February | 62 | 26 | 88 |
2023 January | 54 | 32 | 86 |
2022 December | 59 | 35 | 94 |
2022 November | 35 | 37 | 72 |
2022 October | 42 | 27 | 69 |
2022 September | 40 | 47 | 87 |
2022 August | 60 | 44 | 104 |
2022 July | 32 | 54 | 86 |
2022 June | 27 | 32 | 59 |
2022 May | 82 | 38 | 120 |
2022 April | 72 | 59 | 131 |
2022 March | 70 | 64 | 134 |
2022 February | 85 | 40 | 125 |
2022 January | 77 | 38 | 115 |
2021 December | 54 | 48 | 102 |
2021 November | 50 | 59 | 109 |
2021 October | 55 | 54 | 109 |
2021 September | 50 | 45 | 95 |
2021 August | 52 | 34 | 86 |
2021 July | 51 | 24 | 75 |
2021 June | 54 | 45 | 99 |
2021 May | 44 | 65 | 109 |
2021 April | 111 | 91 | 202 |
2021 March | 56 | 41 | 97 |
2021 February | 66 | 35 | 101 |
2021 January | 62 | 27 | 89 |
2020 December | 42 | 25 | 67 |
2020 November | 46 | 23 | 69 |
2020 October | 56 | 21 | 77 |
2020 September | 28 | 21 | 49 |
2020 August | 32 | 22 | 54 |
2020 July | 33 | 19 | 52 |
2020 June | 43 | 20 | 63 |
2020 May | 26 | 20 | 46 |
2020 April | 25 | 17 | 42 |
2020 March | 26 | 16 | 42 |
2020 February | 8 | 0 | 8 |
2020 January | 1 | 0 | 1 |
2019 December | 4 | 0 | 4 |
2019 September | 4 | 0 | 4 |
2019 June | 2 | 0 | 2 |
2019 May | 2 | 1 | 3 |
2019 March | 2 | 0 | 2 |
2019 January | 2 | 0 | 2 |
2018 December | 2 | 0 | 2 |
2018 October | 2 | 0 | 2 |
2018 September | 3 | 0 | 3 |
2018 February | 26 | 10 | 36 |
2018 January | 39 | 4 | 43 |
2017 December | 47 | 13 | 60 |
2017 November | 69 | 32 | 101 |
2017 October | 6 | 11 | 17 |
2017 September | 3 | 9 | 12 |