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Ocular involvement in a patient with Sweet syndrome: report of a case and review of the literature
Afectación oftalmológica en el contexto de un síndrome de Sweet: comunicación de un caso y revisión de la literatura
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A) Biopsia cutánea que incluye epidermis, dermis e hipodermis. A nivel de la dermis superficial (ver flechas) hay un infiltrado inflamatorio en sábana que se condensa alrededor de los vasos (#) y algunos ductos sudoríparos (*), sin vasculitis. Hematoxilina-eosina,4<span class="elsevierStyleHsp" style=""></span>x. B) El infiltrado inflamatorio de la dermis superficial está compuesto predominantemente por neutrófilos con presencia de leucocitoclastia y ocasionales macrófagos. No hay alteración de la epidermis. Hematoxilina-eosina, 20x.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "E. Guzmán-Almagro, A. Fernández-Hortelano, A. Díaz-Menéndez, J. González-Martín-Moro" "autores" => array:4 [ 0 => array:2 [ "nombre" => "E." "apellidos" => "Guzmán-Almagro" ] 1 => array:2 [ "nombre" => "A." "apellidos" => "Fernández-Hortelano" ] 2 => array:2 [ "nombre" => "A." "apellidos" => "Díaz-Menéndez" ] 3 => array:2 [ "nombre" => "J." 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Guzmán-Almagro, A. Fernández-Hortelano, A. Díaz-Menéndez, J. González-Martín-Moro" "autores" => array:4 [ 0 => array:4 [ "nombre" => "E." "apellidos" => "Guzmán-Almagro" "email" => array:1 [ 0 => "elenaguzmanal@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "A." "apellidos" => "Fernández-Hortelano" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "A." "apellidos" => "Díaz-Menéndez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "J." "apellidos" => "González-Martín-Moro" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Departamento de Oftalmología, Hospital Universitario del Henares, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Departamento de Anatomía Patológica, Hospital Universitario del Henares, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Departamento de Medicina, Universidad Francisco de Vitoria, Madrid, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Afectación oftalmológica en el contexto de un síndrome de Sweet: comunicación de un caso y revisión de la literatura" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 837 "Ancho" => 1250 "Tamanyo" => 344554 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Skin biopsy. A) Skin biopsy including epidermis, dermis and hypodermis. At the level of the superficial dermis (see arrows) an inflammatory sheet infiltrate that condenses around the vessels (#) and some sweat ducts (*) can be observed, without vasculitis. Hematoxylin-eosin,4 x. B) The inflammatory infiltrate of the superficial dermis is composed predominantly of neutrophils with the presence of leukocytoclastia and occasional macrophages. There is no alteration of the epidermis. Hematoxylin-eosin, 20x.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Sweet's syndrome (SS) or acute neutrophilic febrile dermatosis is a rare disease of unknown etiology first described in 1964 by Dr. Robert Sweet. It affects middle-aged patients, with a greater predominance of females in a 3:1 ratio<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a>. It is characterized by the abrupt appearance of painful skin lesions such as reddish blue papules and nodule type lesions. The size of these lesions varies and they can appear in any body region. Histologically the presence of neutrophilic infiltration at the level of the dermis is very typical. Although it is estimated that ocular involvement occurs in one third of cases, very few cases have been published in the ophthalmological literature (only 20 were counted until 2008)<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a>.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinic case report</span><p id="par0010" class="elsevierStylePara elsevierViewall">A 66 year-old woman attended the Emergency Department for conjunctival hyperemia associated with pruritic erythematous lesions accompanied by edema in the facies and trunk of only 24<span class="elsevierStyleHsp" style=""></span>hours of evolution (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A). The previous week she had presented a catarrhal condition. Personal history featured the diagnosis of ulcerative colitis more than 30 years ago in treatment with mesalazine.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Examination showed periorbital edema extending to the upper lip and oral mucosa, even reaching the uvula, without exhibiting respiratory difficulty. Palpable nummular dermal lesions were erythematoedematous, infiltrated, circumferential, painless plaques less than 5<span class="elsevierStyleHsp" style=""></span>cm in diameter that did not disappear under finger opressure. Said lesions were distributed in the malar, chin, cervical, scapular, abdominal and lower limb areas (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B). Treatment with intravenous corticoid was initiated, initially with hydrocortisone and later, after skin biopsy results, with oral prednisone at a dose of 0.5<span class="elsevierStyleHsp" style=""></span>mg/kg body weight per day.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The patient was referred to Ophthalmology to assess eye inflammation. On examination, visual acuity was preserved. Intraocular pressure and funduscopy showed no alterations. The anterior segment exhibited a mixed injection with hemorrhagic characteristics accompanied by chemosis (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>C). Hemorrhagic conjunctivitis was diagnosed in the context of SS and dexamethasone eye drops were prescribed, one drop every 6<span class="elsevierStyleHsp" style=""></span>hours during one week.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Analysis showed mild leukopenia of 2.70x103/μL (3.6 - 10.5) and mild lymphopenia of 0.8x103/μL (1.1 - 4.0). C-reactive protein values were 85.3<span class="elsevierStyleHsp" style=""></span>mg/l (0.0 - 5.0).</p><p id="par0030" class="elsevierStylePara elsevierViewall">At the histopathological level, a neutrophilic dermatitis with leukocytoclastia and edema in papillary dermis compatible with SS was evidenced. As observed in the present case, inflammatory infiltrates can be slightly condensed around the vessels and sweat ducts, without producing vasculitis (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>A). <a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>B shows an infiltration predominantly of neutrophil cells with leukocytoclastia and some isolated macrophages (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>B).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">The hemorrhagic conjunctivitis, as well as the systemic clinic, resolved almost completely after a week, and the analysis values were normalized. As a result of the improvement, oral corticosteroids were administered until the end of the one-month treatment cycle. In the last six months the patient did not present with any recurrence.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">SS is a rare disease that causes painful, erythematous lesions of sudden onset accompanied by fever, malaise, arthralgia or headache and an elevation of acute phase reactants (leukocytosis with neutrophilia, ESV and C-reactive protein).</p><p id="par0045" class="elsevierStylePara elsevierViewall">Its etiology is unknown but there could be an immune dysfunction. It appears that inadequate signaling by cytokines leads to recruitment and activation of neutrophils. This dysfunctional response of neutrophils intoxicates the tissues, producing the characteristic manifestations.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Although it has traditionally been estimated that one third of SS cases present eye injuries, the fact is that the percentage is probably lower. Of the 136 cases described, only 24 (18%) exhibited ophthalmological manifestations. The most common compromise involves the anterior segment, although some cases with posterior pole compromise have also been described. Expressions are highly variable and may produce conjunctivitis, episcleritis, limb nodules, uveitis, dacryoadenitis, palpebral inflammation, peripheral ulcerative keratitis, glaucoma, orbital inflammation<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> and even vasculitis and retinitis<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a>. Conventionally, the pathologies most involved have been conjunctivitis and episcleritis<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3,5</span></a> but recent series point to scleritis as the most frequent<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a>.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Ocular injury may occur uni- or bilaterally. Its appearance can be simultaneously with the dermal alterations, days later or in the absence thereof.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Organs such as bone, lung, liver, spleen and brain may be involved, and like the eye, their involvement in this syndrome may be synchronous, before or after dermal infiltration<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a>.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Skin lesion sizes range between 0.5 and 12<span class="elsevierStyleHsp" style=""></span>cm and tend to compromise the upper hemicorpus (face, neck, trunk and beginning of limbs), although lesions on fingers, wrists, hands or legs have also been described<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a>.</p><p id="par0070" class="elsevierStylePara elsevierViewall">Neutrophilic dermatoses are classified into 4 types according to the depth of the involvement: epidermis (giving rise to pustules), dermis (papules and plaques) and deep dermis (ulcers). SS corresponds to the variant that compromise the dermis. Erythema nodosum can be associated with SS but is not a neutrophilic dermatosis.</p><p id="par0075" class="elsevierStylePara elsevierViewall">A diagnosis of SS must fulfil 4 criteria, two regarded as major and two minor (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>).</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0080" class="elsevierStylePara elsevierViewall">When SS is diagnosed, it is necessary to rule out any underlying malignant process, infection or systemic disease since there is a greater frequency of recurrences in these cases.</p><p id="par0085" class="elsevierStylePara elsevierViewall">According to its origin, SS can be classified into 3 types:<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">-</span><p id="par0090" class="elsevierStylePara elsevierViewall">Classic or idiopathic: the most frequent type and the one with the highest ocular compromise. It is associated with respiratory or gastrointestinal tract infection, inflammatory digestive disease or pregnancy. This is the type exhibited by the present patient.</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">-</span><p id="par0095" class="elsevierStylePara elsevierViewall">Associated with underlying malignant pathology, which accounts for 15-20% of cases, mainly associated with acute myeloid leukemia and myelodysplasia. It can also be associated with solid tumors of the genitourinary, gastrointestinal tracts or the breast. In the event of recurrent ophthalmological lesions in isolation, the range of tumors and infections to be considered would have to be broadened.</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">-</span><p id="par0100" class="elsevierStylePara elsevierViewall">Induced by various drugs: antibiotics (minocycline, nitrofurantoin, trimetropim-sulfamethoxazole), antihypertensives, anti-epileptics, granulocyte colony stimulating factor (G-CSF), oral contraceptives and retinoids.</p></li></ul></p><p id="par0105" class="elsevierStylePara elsevierViewall">Although there are no clinical guidelines for treatment, the use of oral steroids at initial doses of 0.5-1.5<span class="elsevierStyleHsp" style=""></span>mg/kg/weight seems to be most indicated. The response is very good and is in fact considered a diagnostic criterion. In drug-induced SS, the inducing drug should be withdrawn if possible. Other drugs have also been used, although with less experience, such as: cyclosporine,<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> colchicine, indomethacin, dapsone<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> and potassium iodide. Topical or intralesional use of steroids does not appear to provide any added benefit to systemic treatment<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a>.</p><p id="par0110" class="elsevierStylePara elsevierViewall">A biopsy of the eye tissue is not considered necessary for the diagnosis of this entity, although in the cases in which it has been performed, a histopathology similar to the dermatological picture is evident.</p><p id="par0115" class="elsevierStylePara elsevierViewall">A very characteristic histopathological feature is the presence of a dense neutrophilic infiltrate with leukocytoclastia in the cutaneous reticular dermis<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a>, although macrophages, eosinophils and lymphocytes can also be observed<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a>.</p><p id="par0120" class="elsevierStylePara elsevierViewall">In some occasions the inflammatory infiltration is so intense that it compromises the papillary dermis inducing changes at the level of the epidermis, posing a differential diagnosis with dermatitis herpetiformis<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a>. However, in the present case the epidermis was respected.</p><p id="par0125" class="elsevierStylePara elsevierViewall">From the anatomopathological point of view, the main differential diagnoses are<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a>:<ul class="elsevierStyleList" id="lis0010"><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">-</span><p id="par0130" class="elsevierStylePara elsevierViewall">Leukocytoclastic vasculitis, whether or not the presence of vascular lesion is key to its diagnosis.</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">-</span><p id="par0135" class="elsevierStylePara elsevierViewall">The facial granuloma usually presents a similar picture with less necrosis and more inflammatory infiltration of eosinophils.</p></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">-</span><p id="par0140" class="elsevierStylePara elsevierViewall">The presence of ulcers helps distinguish pyoderma gangrenosum from SS.</p></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">-</span><p id="par0145" class="elsevierStylePara elsevierViewall">Infectious condition that can also induce dense neutrophilic infiltration.</p></li><li class="elsevierStyleListItem" id="lsti0040"><span class="elsevierStyleLabel">-</span><p id="par0150" class="elsevierStylePara elsevierViewall">At the level of skin biopsy, it is not possible to differentiate an SS from Bichet's disease, as the histopathological findings may be overlapping. It has been suggested that SS and Beçhet's disease coexist in the same patient<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a>, with the possibility that the clinical expressions constitute a continuous spectrum in these two pathologies<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a>.</p></li></ul></p><p id="par0155" class="elsevierStylePara elsevierViewall">In conclusion, a classic SS condition is presented, with the most frequent ocular manifestation being conjunctivitis. It must be taken into account that this pathology is accompanied by ophthalmological compromise in one third of patients. Therefore, it is important for the ophthalmologist to be familiar with the broad range of ocular manifestations that can be associated with this syndrome.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflict of interests</span><p id="par0160" class="elsevierStylePara elsevierViewall">No conflict of interests was declared by the authors.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres1407065" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1287295" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1407064" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1287294" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Clinic case report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conflict of interests" ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2020-04-14" "fechaAceptado" => "2020-06-10" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1287295" "palabras" => array:3 [ 0 => "Sweet syndrome" 1 => "Acute febrile neutrophilic dermatosis" 2 => "Conjunctivitis" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1287294" "palabras" => array:3 [ 0 => "Síndrome de Sweet" 1 => "Dermatosis neutrofílica febril aguda" 2 => "Conjuntivitis" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">The case presented is a 66-year-old woman who attended the emergency department due to skin lesions on the limbs, facial oedema, and eye redness accompanied by haemorrhagic conjunctivitis. The symptoms resolved after one week of systemic steroid treatment. Skin biopsy confirmed Sweet syndrome.</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Sweet syndrome is rare disorder and unknown by most ophthalmologists despite its frequent ophthalmological manifestations. Ocular involvement is present in one third of patients, with episcleritis and conjunctivitis being the most repeated. Pathology findings confirm the diagnosis which is also characterised by a rapid response to systemic corticosteroids.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Presentamos el caso clínico de una mujer de 66 años que acudió a urgencias por lesiones cutáneas en miembros tanto superiores como inferiores, edema facial y enrojecimiento ocular, acompañados de conjuntivitis hemorrágica. Tras pautar corticoides sistémicos el cuadro se resolvió en una semana. La biopsia cutánea confirmó que se trataba de un síndrome de Sweet.</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">El síndrome de Sweet es una enfermedad infrecuente y desconocida para la mayor parte de los oftalmólogos a pesar de cursar con manifestaciones oftalmológicas. La afectación ocular está presente en un tercio de los pacientes, siendo los más usuales la epiescleritis y la conjuntivitis. El diagnóstico de confirmación es histopatológico y se caracteriza por una rápida respuesta a los corticoides sistémicos.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Guzmán-Almagro E, Fernández-Hortelano A, Díaz-Menéndez A, González-Martín-Moro J. Afectación oftalmológica en el contexto de un síndrome de Sweet: comunicación de un caso y revisión de la literatura. Arch Soc Esp Oftalmol. 2020;95:550–554.</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" => 1257 "Ancho" => 1500 "Tamanyo" => 190506 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">A) facial, B) trunk, and C) ocular compromise.</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" => 837 "Ancho" => 1250 "Tamanyo" => 344554 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Skin biopsy. A) Skin biopsy including epidermis, dermis and hypodermis. At the level of the superficial dermis (see arrows) an inflammatory sheet infiltrate that condenses around the vessels (#) and some sweat ducts (*) can be observed, without vasculitis. Hematoxylin-eosin,4 x. B) The inflammatory infiltrate of the superficial dermis is composed predominantly of neutrophils with the presence of leukocytoclastia and occasional macrophages. There is no alteration of the epidermis. Hematoxylin-eosin, 20x.</p>" ] ] 2 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Major criteria</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Sudden onset of painful, erythematous plaques or nodules \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Typical pathological anatomy: dense neutrophilic infiltrate in dermis with no evidence of vasculitis \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleItalic">Minor criteria</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Fever > 38<span class="elsevierStyleHsp" style=""></span>°C, malaise \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Association with hematological or visceral malignancy, inflammatory disease (ulcerative colitis, Crohn's disease) or pregnancy or preceded by upper respiratory tract infection, gastrointestinal infection or vaccination \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Very good response to treatment with systemic steroids \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Analytical alterations at debut (fulfilling 3 of the 4): \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Globular sedimentation rate > 20 mm/hour \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>High C-reactive protein values \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Leukocytes > 8x109/L \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>Neutrophils>70% \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2413033.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Diagnostic criteria. The patient must meet two major and at least two minor criteria.</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" => "Sweet Syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "P. Vashisht" 1 => "P. Bansal" 2 => "A. Goyal" 3 => "M.P. 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