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González Fernández, S. Requena López, F. Valdés Pineda" "autores" => array:3 [ 0 => array:2 [ "nombre" => "D. González" "apellidos" => "Fernández" ] 1 => array:2 [ "nombre" => "S. Requena" "apellidos" => "López" ] 2 => array:2 [ "nombre" => "F. 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Benavente Villegas, J.M. Sánchez Motilla, N. Rausell Fontestad" "autores" => array:3 [ 0 => array:4 [ "nombre" => "F.C. Benavente" "apellidos" => "Villegas" "email" => array:1 [ 0 => "felipecbv@gmail.com" ] "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" => "J.M. Sánchez" "apellidos" => "Motilla" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "N. Rausell" "apellidos" => "Fontestad" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario Doctor Peset, Valencia, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitario Doctor Peset, Valencia, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Vasculitis con lesiones en cuero cabelludo como única manifestación cutánea" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 703 "Ancho" => 1250 "Tamanyo" => 149810 ] ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">The patient was a 65-year-old man (a nonsmoker) with a past history of nasal polyps and late-onset bronchial asthma that was very difficult to control. He had been hospitalized multiple times and was a long-term user of oral prednisone at a dosage of 15<span class="elsevierStyleHsp" style=""></span>mg daily. In April 2015, the patient was enrolled by the pulmonology department in a clinical trial that compared lebrikizumab to placebo; the first step was to gradually reduce his prednisone dosage to 2.5<span class="elsevierStyleHsp" style=""></span>mg daily. A week after this dosage was reached, the patient had an asthma exacerbation and very pruritic lesions appeared on the scalp, accompanied 15 days later by paresthesia, pain, and complete hemiplegia of the lower right limb.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed excoriated, erythematous, purpuric papules and plaques, including some with central ulceration (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). No other associated lesions were observed.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Histologic examination revealed a cutaneous ulcer with necrotizing vasculitis and a mixed neutrophilic and eosinophilic infiltrate in perivascular and interstitial areas (<a class="elsevierStyleCrossRefs" href="#fig0010">Figs. 2 and 3</a>). Cultures, Gram stain, and direct immunofluorescence were negative.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Blood tests revealed eosinophilia (19 620 eosinophils/μl; 72%) and perinuclear antineutrophil cytoplasmic antibodies (pANCA) (34<span class="elsevierStyleHsp" style=""></span>IU/mL; <<span class="elsevierStyleHsp" style=""></span>5 IU/mL is normal).</p><p id="par0025" class="elsevierStylePara elsevierViewall">Electromyography revealed mononeuritis multiplex in the right leg.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Chest radiography revealed minimal bilateral pleural effusion.</p><p id="par0035" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What Is Your Diagnosis?</span></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0040" class="elsevierStylePara elsevierViewall">Eosinophilic granulomatosis with polyangiitis (EGPA).</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0045" class="elsevierStylePara elsevierViewall">Treatment was started with intravenous methylprednisolone at a dosage of 1<span class="elsevierStyleHsp" style=""></span>g daily for 5 days, followed by oral prednisone at a dosage of 1<span class="elsevierStyleHsp" style=""></span>mg/kg/d. Adequate response was achieved, except in the neurologic manifestations. The patient was then removed from the clinical trial and treatment was started with rituximab. Significant improvement was observed at subsequent follow-up visits.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0050" class="elsevierStylePara elsevierViewall">EGPA, previously known as Churg-Strauss syndrome, is a multisystem disease that occurs with equal frequency in both sexes and is characterized by allergic rhinitis, asthma, and severe eosinophilia.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a> It is classified in the group of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides affecting small and medium-sized vessels and is the least common of the 3 entities included in this group.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Given the presence of ANCA, the alteration of type 1 and type 2 T-helper cells, the decrease in regulatory T-cells, and eosinophilia caused by both increased synthesis and decreased apoptosis, it has been suggested that EGPA may have an immunologic pathogenesis.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">2,3</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Several authors have reported EGPA in systemic-corticosteroid-dependent asthmatic patients following a reduction in corticosteroid dosage and the start of treatment with leukotriene modifiers and/or omalizumab.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">4,5</span></a> These authors concluded that the onset of the disease was not related to the start of treatment with new drugs but to the reduction in corticosteroid dosage, and that therefore there was no causal relationship but rather an unmasking of underdiagnosed EGPA.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">4,5</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">EGPA develops in 3 phases: a prodromal phase, characterized by underlying atopy and late-onset asthma that is difficult to control; an eosinophilic phase, characterized by prominent blood eosinophilia; and finally a vasculitic phase, characterized by the development of necrotizing vasculitis of small and medium-sized vessels, an eosinophilic infiltrate, and in many cases vascular and extravascular granulomatosis. The vasculitic phase is characterized by constitutional symptoms and multisystemic manifestations such as mononeuritis multiplex, microhematuria, and cardiac failure, which is the most common cause of death.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">6</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Cutaneous manifestations—typical of the vasculitic phase—tend to include subcutaneous nodules, palpable purpura, and hemorrhagic maculopapular lesions. These lesions occur most frequently on the lower limbs; however, lesions on the scalp are distinctive, although not pathognomonic.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">6</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Between 40% and 60% of patients with EGPA are positive for ANCA, typically pANCA, although the presence of these antibodies does not predict activity and should not be taken into account in follow-up.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">6</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">There is no specific diagnostic test for EGPA; diagnosis relies on preestablished criteria and evidence of vasculitis in the affected organ.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">6</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Treatment involves systemic corticosteroids, sometimes in association with cyclophosphamide if cardiac, renal, or central nervous system involvement is present.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">6,7</span></a> Rituximab, mepolizumab, and omalizumab have been used in refractory cases, with disparate results.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">7</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">Differential diagnosis should include other entities characterized by angiolymphoid hyperplasia with eosinophilia as well as hypereosinophilic syndrome, as these are typically not associated with asthma or histopathologic findings indicative of vasculitis.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">2,6</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">In conclusion, we postulate that the reduction in corticosteroid dosage was the factor that triggered systemic manifestations in our patient, since these drugs likely masked the EGPA, which had not yet entered the vasculitic phase. We highlight the characteristic presentation of vasculitic lesions on the scalp. In patients with asthma, severe eosinophilia, systemic manifestations, and skin lesions, early histologic diagnosis is essential in order to begin appropriate treatment.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of Interest</span><p id="par0100" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Medical History" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Physical Examination" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Histopathology" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Additional Tests" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Diagnosis" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Clinical Course and Treatment" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Comment" ] 7 => array:2 [ "identificador" => "sec0040" "titulo" => "Conflicts of Interest" ] 8 => 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: Villegas FCB, Motilla JMS, Fontestad NR. Vasculitis con lesiones en cuero cabelludo como única manifestación cutánea. Actas Dermosifiliogr. 2018;109:175–176.</p>" ] ] "multimedia" => array:3 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 703 "Ancho" => 1250 "Tamanyo" => 149810 ] ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 917 "Ancho" => 688 "Tamanyo" => 228406 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin-eosin, original magnification ×4.</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" => 917 "Ancho" => 688 "Tamanyo" => 262203 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin-eosin, original magnification ×40.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0040" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Churg-Strauss syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "C. 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Year/Month | Html | Total | |
---|---|---|---|
2024 November | 16 | 10 | 26 |
2024 October | 150 | 50 | 200 |
2024 September | 139 | 37 | 176 |
2024 August | 235 | 60 | 295 |
2024 July | 232 | 42 | 274 |
2024 June | 183 | 45 | 228 |
2024 May | 195 | 44 | 239 |
2024 April | 142 | 38 | 180 |
2024 March | 191 | 29 | 220 |
2024 February | 219 | 32 | 251 |
2024 January | 174 | 33 | 207 |
2023 December | 195 | 20 | 215 |
2023 November | 202 | 20 | 222 |
2023 October | 145 | 36 | 181 |
2023 September | 145 | 29 | 174 |
2023 August | 135 | 21 | 156 |
2023 July | 157 | 47 | 204 |
2023 June | 111 | 21 | 132 |
2023 May | 119 | 27 | 146 |
2023 April | 110 | 26 | 136 |
2023 March | 149 | 46 | 195 |
2023 February | 106 | 36 | 142 |
2023 January | 88 | 34 | 122 |
2022 December | 81 | 44 | 125 |
2022 November | 54 | 28 | 82 |
2022 October | 63 | 28 | 91 |
2022 September | 39 | 56 | 95 |
2022 August | 25 | 27 | 52 |
2022 July | 30 | 42 | 72 |
2022 June | 25 | 33 | 58 |
2022 May | 78 | 46 | 124 |
2022 April | 116 | 46 | 162 |
2022 March | 121 | 80 | 201 |
2022 February | 115 | 30 | 145 |
2022 January | 136 | 54 | 190 |
2021 December | 71 | 45 | 116 |
2021 November | 86 | 54 | 140 |
2021 October | 91 | 73 | 164 |
2021 September | 54 | 64 | 118 |
2021 August | 56 | 57 | 113 |
2021 July | 54 | 42 | 96 |
2021 June | 49 | 33 | 82 |
2021 May | 68 | 65 | 133 |
2021 April | 90 | 129 | 219 |
2021 March | 76 | 41 | 117 |
2021 February | 91 | 49 | 140 |
2021 January | 39 | 53 | 92 |
2020 December | 50 | 58 | 108 |
2020 November | 54 | 49 | 103 |
2020 October | 19 | 16 | 35 |
2020 September | 45 | 27 | 72 |
2020 August | 28 | 35 | 63 |
2020 July | 37 | 26 | 63 |
2020 June | 43 | 38 | 81 |
2020 May | 22 | 17 | 39 |
2020 April | 24 | 18 | 42 |
2020 March | 36 | 14 | 50 |
2020 February | 4 | 0 | 4 |
2020 January | 4 | 0 | 4 |
2019 December | 8 | 0 | 8 |
2019 November | 4 | 0 | 4 |
2019 September | 11 | 0 | 11 |
2019 August | 4 | 0 | 4 |
2019 July | 4 | 0 | 4 |
2019 June | 4 | 0 | 4 |
2019 May | 5 | 0 | 5 |
2019 April | 2 | 0 | 2 |
2019 March | 2 | 0 | 2 |
2019 February | 2 | 0 | 2 |
2018 December | 5 | 0 | 5 |
2018 November | 1 | 0 | 1 |
2018 September | 3 | 0 | 3 |
2018 April | 1 | 0 | 1 |
2018 February | 3 | 9 | 12 |
2018 January | 5 | 10 | 15 |