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true "cabecera" => "<span class="elsevierStyleTextfn">Letter to the Editor</span>" "titulo" => "Belimumab in systemic lupus erythematosus: Experience in clinical practice settings in a regional hospital" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "188" "paginaFinal" => "189" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Belimumab en lupus eritematoso sistémico: experiencia en práctica clínica en un hospital comarcal" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Leyre Riancho-Zarrabeitia, Ignacio Villa Blanco, Montserrat Santos-Gómez, Elena Aurrecoechea" "autores" => array:4 [ 0 => array:2 [ "nombre" => "Leyre" "apellidos" => "Riancho-Zarrabeitia" ] 1 => array:2 [ "nombre" => "Ignacio" "apellidos" => "Villa Blanco" ] 2 => array:2 [ "nombre" => "Montserrat" "apellidos" => "Santos-Gómez" ] 3 => 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"<span class="elsevierStyleItalic">Dear Editor,</span>" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "189" "paginaFinal" => "190" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "María Isabel García Briz, Amelia Prats Máñez, Ramón García Ruiz, Almudena Mateu Puchades" "autores" => array:4 [ 0 => array:4 [ "nombre" => "María Isabel" "apellidos" => "García Briz" "email" => array:1 [ 0 => "mariwelchy@gmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "Amelia" "apellidos" => "Prats Máñez" ] 2 => array:2 [ "nombre" => "Ramón" "apellidos" => "García Ruiz" ] 3 => array:2 [ "nombre" => "Almudena" "apellidos" => "Mateu Puchades" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Dermatología, Hospital Universitario Doctor Peset, Valencia, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Vasculopatía livedoide en una paciente con penfigoide ampolloso y síndrome de Sjögren primario" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 585 "Ancho" => 750 "Tamanyo" => 38420 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Clinical image of the skin lesions.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Livedoid vasculopathy is a chronic, recurrent and painful skin disease that usually affects the lower limbs. It was first described by Bard and Winkelmann in 1967,<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> as a vaso-occlusive disorder affecting the small vessels of the dermis.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of an 82-year-old woman under dermatological follow-up for bullous pemphigoid, which was stable on low doses of prednisone. Of interest in her personal history was a prior diagnosis of primary Sjögren's syndrome with positive anti-Ro antibodies, meeting the diagnostic criteria set established by Vitali et al.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">During a check-up visit, coinciding with more xerophthalmia and xerostomia than usual, reticulated erythematous macules of livedoid appearance together with painful ulcerated nodules and other areas of atrophie blanche were observed on both lower limbs bilaterally and symmetrically (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Due to a clinical suspicion of livedoid vasculopathy, a diagnostic biopsy was undertaken showing thickening and hyalinisation of the vessel walls, with no inflammatory component.</p><p id="par0025" class="elsevierStylePara elsevierViewall">A complete blood test highlighted: anaemia with a haemoglobin of 10.4<span class="elsevierStyleHsp" style=""></span>g/dl, positive ANA 1/1280 with speckled cytoplasmic patterns, positive anti-SSA/Ro antibodies (>240.0<span class="elsevierStyleHsp" style=""></span>U/ml), reduced C3 and C4 (43 and 3<span class="elsevierStyleHsp" style=""></span>mg/dl, respectively). The remaining parameters showed no anomalies.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Therefore, given the characteristic clinical skin symptoms and compatible histological findings, the diagnosis of livedoid vasculopathy was confirmed, and possible haematological alterations that would explain a prothrombotic condition were ruled out.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Treatment was started with pentoxifylline 400<span class="elsevierStyleHsp" style=""></span>mg every 8<span class="elsevierStyleHsp" style=""></span>h, with 100<span class="elsevierStyleHsp" style=""></span>mg of aspirin daily, achieving a good response and gradual resolution of the lesions.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Livedoid vasculopathy is a rare, chronic and painful disease, characterised by the presence of macules or purpuric papules and plaques with a tendency to form irregular ulcers that develop into star-shaped atrophic scars and peripheral hyperpigmentation, described as atrophie blanche.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> It usually affects the lower limbs, with a bilateral, symmetrical distribution. It is characteristically, although not always, associated with livedo reticularis.</p><p id="par0045" class="elsevierStylePara elsevierViewall">It can manifest at any time of life, and is more frequent in young women, with a 3:1 ratio over males.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> However, our patient was older than the average.</p><p id="par0050" class="elsevierStylePara elsevierViewall">The most frequent histopathological finding is hyalinising vascular changes of the inner layers of the dermal vessels, generally with little inflammation, together with thrombosis inside the blood vessels.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">4</span></a> These signs enable the diagnosis to be confirmed, and other processes that present with similar skin symptoms to be ruled out.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The condition's aetiopathogenesis remains unknown. However, the presence of thrombophilic alterations is considered increasingly relevant, and complementary tests are needed to rule out prothrombotic conditions.</p><p id="par0060" class="elsevierStylePara elsevierViewall">In turn, it has been related to systemic diseases, such as scleroderma, systemic lupus erythematosus, rheumatoid arthritis, cryoglobulinaemia, and mixed connective tissue disease.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">6,7</span></a> However, only one case relating to Sjögren's syndrome<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> has been published, and its simultaneous coexistence with two autoimmune conditions has not been recorded to date.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">To conclude, we present a case of livedoid vasculopathy in a patient with bullous pemphigoid and primary Sjögren's syndrome with positive anti-Ro antibodies and complement consumption. The co-existence of this disease with both autoimmune conditions reinforces the hypothesis that there is an association with systemic diseases that can be aggressive to the endothelium, triggering the onset of this vaso-occlusive disease. However, studies with a larger number of patients are necessary to obtain definitive results.</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: García Briz MI, Prats Máñez A, García Ruiz R, Mateu Puchades A. Vasculopatía livedoide en una paciente con penfigoide ampolloso y síndrome de Sjögren primario. Reumatol Clin. 2020;16:189–190.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 585 "Ancho" => 750 "Tamanyo" => 38420 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Clinical image of the skin lesions.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:9 [ 0 => array:3 [ "identificador" => "bib0050" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Livedo vasculitis: segmental hyalinisation of the dermis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "J.W. Bard" 1 => "R.K. 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