Images in Paediatrics
Cutaneous polyarteritis nodosa: it is not always erythema nodosum
Panarteritis nodosa cutánea: no todo es eritema nodoso
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A) Lesión en fase activa. Placa eritematosa, de bordes mal definidos, caliente, con componente central purpúrico-equimótico y livedo reticularis, indicativa de vasculitis. Asocia hiperestesia y dolor a la palpación y a la movilización del pie. En la planta del mismo pie presenta nódulo de 1<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>1<span class="elsevierStyleHsp" style=""></span>cm aproximadamente, que asocia signos inflamatorios externos (no descrita en la imagen). Presenta además placa de 5<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>3<span class="elsevierStyleHsp" style=""></span>cm aproximadamente, con extensión desde tercio inferior de pierna izquierda a maléolo externo, mal definida, indurada, con aumento de temperatura, dolorosa a la palpación (no visualizable en la imagen). B) Lesión en el pie izquierdo, en fase de resolución. Eritema en el dorso del pie y alodinia residual. Desaparición del componente purpúrico-equimótico y de livedo reticularis. C y D) Evolución de la lesión nodular a nivel de 2.° dedo de la mano derecha.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Nicholas Bello, Rocio Galindo Zavala, Isabel Leiva Gea, Ángel Vera Casaño" "autores" => array:4 [ 0 => array:2 [ "nombre" => "Nicholas" "apellidos" => "Bello" ] 1 => array:2 [ "nombre" => "Rocio" "apellidos" => "Galindo Zavala" ] 2 => array:2 [ "nombre" => "Isabel" "apellidos" => "Leiva Gea" ] 3 => array:2 [ "nombre" => "Ángel" "apellidos" => "Vera Casaño" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2341287924000814" "doi" => "10.1016/j.anpede.2024.03.026" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2341287924000814?idApp=UINPBA00005H" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1695403320301958?idApp=UINPBA00005H" "url" => "/16954033/0000009400000006/v1_202106020556/S1695403320301958/v1_202106020556/es/main.assets" ] ] "itemSiguiente" => array:19 [ "pii" => "S2341287923002818" "issn" => "23412879" "doi" => "10.1016/j.anpede.2020.07.026" "estado" => "S300" "fechaPublicacion" => "2024-04-01" "aid" => "2947" "copyright" => "Asociación Española de Pediatría" "documento" => "article" "crossmark" => 1 "subdocumento" => "sco" "cita" => "An Pediatr (Barc). 2024;100:e13-e14" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "en" => array:11 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Images in Paediatrics</span>" "titulo" => "Periportal cuffing as an ultrasound sign of myeloproliferative syndrome" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "e13" "paginaFinal" => "e14" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Manguito periportal como signo ecográfico de síndrome mieloproliferativo" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1041 "Ancho" => 2092 "Tamanyo" => 207878 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0120" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Oblique longitudinal plane of the left hypochondrium obtained with a convex probe at the level of the epigastrium, showing: a) Hepatic parenchyma at the level of the left lobe (LHI) with transversal sections of echo-poor periportal haloes of larger (M) and smaller (m) size; b) Enlargement of previous image with measurement of the diameter of one of the periportal haloes. 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nodosa: it is not always erythema nodosum" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "e10" "paginaFinal" => "e12" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Nicholas Bello, Rocio Galindo Zavala, Isabel Leiva Gea, Ángel Vera Casaño" "autores" => array:4 [ 0 => array:4 [ "nombre" => "Nicholas" "apellidos" => "Bello" "email" => array:1 [ 0 => "nicholasbello90@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" => "Rocio" "apellidos" => "Galindo Zavala" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Isabel" "apellidos" => "Leiva Gea" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "Ángel" "apellidos" => "Vera Casaño" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Reumatología Pediatrica, Hospital Materno-Infantil de Málaga, Málaga, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Serivicio de Endocrinología Pediatrica, Hospital Materno-Infantil de Málaga, Málaga, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Servicio de Dermatología Pediatrica, Hospital Materno-Infantil de Málaga, Málaga, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Panarteritis nodosa cutánea: no todo es eritema nodoso" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 3334 "Ancho" => 2507 "Tamanyo" => 464907 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0125" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Evolution of the lesion in the dorsum of the right foot. (A) Active phase. Erythematous plaque with poorly defined borders, warm to the touch, with central purpuric bruise-like component and livedo reticularis, indicative of vasculitis. It was associated with hyperaesthesia and pain on palpation and movement of the foot. In the sole of the same foot, there was a nodule measuring approximately 1<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>1<span class="elsevierStyleHsp" style=""></span>cm with external signs of inflammation (not featured in image). There was also a plaque measuring approximately 5<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>3<span class="elsevierStyleHsp" style=""></span>cm extending from the lower third of the left leg to the lateral malleolus, indurated, with poorly defined borders, warm to the touch and painful on palpation (not featured in image). (B) Lesion in remission. Erythema in the dorsum of the foot and residual allodynia. Resolution of the purpuric component and livedo reticularis. (C) and (D) Evolution of the nodular lesion in the 2nd finger of the right hand.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A girl aged 12 years presented with oral aphthae and bilateral cutaneous lesions in the extremities (<a class="elsevierStyleCrossRefs" href="#fig0005">Figs. 1 and 2</a>) suggestive of vasculitis (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A) and pain limiting movement of the lower right extremity of 6 days’s duration.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">The only relevant history was an episode of streptococcal pharyngitis 3 weeks prior, and erythema nodosum was initially suspected. Given the severity of the pain, which could only be alleviated with morphine, the location of the lesions and the presence of livedo reticularis, a lesion biopsy specimen was obtained, revealing vascular wall necrosis and thrombosis in medium-sized vessels, compatible with polyarteritis nodosa (PAN). The CT angiogram of the chest and abdomen angio-TC toraco-abdominal, acute phase reactants, autoimmune testing (including antineutrophil cytoplasmic and antiphospholipid antibodies) and serologic tests for infection were all normal.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The patient received a diagnosis of cutaneous PAN (cPAN) and started treatment with prednisone (2<span class="elsevierStyleHsp" style=""></span>mg/kg/day). She had a partial response, leading to addition of intravenous immunoglobulin (IVIG, at a dose of 2<span class="elsevierStyleHsp" style=""></span>g/kg over 48<span class="elsevierStyleHsp" style=""></span>h) and colchicine. This achieved an excellent outcome. It allowed discontinuation of steroid therapy within 2 months and colchicine at 1<span class="elsevierStyleHsp" style=""></span>year, without recurrence. The only remaining sequela is hyperaesthesia in the dorsum of the foot.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Cutaneous PAN is a necrotising vasculitis involving medium-sized vessels<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a> that is frequently preceded by streptococcal pharyngitis<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> of which fewer than 100 cases have been described in the paediatric population.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Its natural history consists in recurrent attacks characterised by the development of painful subcutaneous nodules in the extremities, occasionally with fever, articular involvement, peripheral neuropathy or myalgia.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> The prognosis is good, and progression to systemic disease is extremely rare.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a> The most frequently used treatments are steroid therapy, colchicine, IVIG, methotrexate, mycophenolate and azathioprine,<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> although there are no studies demonstrating their efficacy.</p><p id="par0030" class="elsevierStylePara elsevierViewall">In conclusion, cPAN is a form of vasculitis that is infrequent in the paediatric age group and that should be considered in the diagnosis of cutaneous nodules with atypical localization or associated with livedo reticularis. The diagnosis is confirmed by histopathology,<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> and there are various treatment options that may be useful.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2020-03-26" "fechaAceptado" => "2020-06-04" "multimedia" => array:2 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 3334 "Ancho" => 2507 "Tamanyo" => 464907 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0125" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Evolution of the lesion in the dorsum of the right foot. (A) Active phase. Erythematous plaque with poorly defined borders, warm to the touch, with central purpuric bruise-like component and livedo reticularis, indicative of vasculitis. It was associated with hyperaesthesia and pain on palpation and movement of the foot. In the sole of the same foot, there was a nodule measuring approximately 1<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>1<span class="elsevierStyleHsp" style=""></span>cm with external signs of inflammation (not featured in image). There was also a plaque measuring approximately 5<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>3<span class="elsevierStyleHsp" style=""></span>cm extending from the lower third of the left leg to the lateral malleolus, indurated, with poorly defined borders, warm to the touch and painful on palpation (not featured in image). (B) Lesion in remission. Erythema in the dorsum of the foot and residual allodynia. Resolution of the purpuric component and livedo reticularis. (C) and (D) Evolution of the nodular lesion in the 2nd finger of the right hand.</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" => 1094 "Ancho" => 2507 "Tamanyo" => 181120 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0130" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Nodular lesions at (A) pretibial level, (B) right elbow and (C) 5th finger of left hand.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:3 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The changing face of polyarteritis nodosa and necrotizing vasculitis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "S. 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| Year/Month | Html | Total | |
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