Images in Clinical Rheumatology
Kikuchi Fujimoto's Disease Appearing as Systemic Lupus Erythematosus
Inicio de lupus eritematoso sistémico como enfermedad de Kikuchi-Fujimoto
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Abundant mucin deposits in the reticular dermis (lower left) which are more evident with a colloidal iron stain (lower right).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Kikuchi–Fujimoto's disease (KFD) is a rare, benign and self-limited disease which on rare occasion may be associated with systemic lupus erythematosus (SLE), and may be present before, after or simultaneously. We analyze the case which demonstrates the relationship between both diseases.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Case Description</span><p id="par0010" class="elsevierStylePara elsevierViewall">A 22-year-old woman with no personal history came to the hospital due to fever and cervical lateral adenopathy which started 15 days prior. Laboratory tests show leukopenia 2800 per mm<span class="elsevierStyleSup">3</span> (with no lymphopenia), normocytic, normochromic anemia (hemoglobin 11<span class="elsevierStyleHsp" style=""></span>g/dl, hematocrit 34%, mean corpuscular volume 85.9, mean corpuscular hemoglobin 28.6<span class="elsevierStyleHsp" style=""></span>pg) and ESR 85<span class="elsevierStyleHsp" style=""></span>mm/h. The blood chemistry and urine sediment shows no abnormalities. HIV, B and C hepatitis, toxoplasma, rubeola, Epstein–Barr virus and cytomegalovirus were negative. Blood cultures and Mantoux tests were negative. Autoimmunity tests showed positive ANA with a 1/320 titer (homogeneous pattern), negative anti-DNA, negative rheumatoid factor and normal complement levels. Thorax and abdominal CT showed multiple cervical and inguinal adenopathies. A bone marrow aspirate-biopsy was performed, as well as cultures, which were all negative. A cervical lymph node biopsy was performed, showing paracortical necrosis with cell detritus and numerous hystiocytes, compatible with necrotizing lymphadenitis or KFD (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). In the following years the patient remained stable, with no further episodes of fever, although she did present self-limited cervical adenopathy. She repeatedly presented painful oral ulcers and further analysis confirmed leukopenia (without lymphopenia). Three years later she presented painful erythematous nodules on the forehead and cheeks (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Laboratory tests revealed positive ANA with a titer of 1/160 with a homogeneous pattern and low complement levels. A skin biopsy was compatible with discoid lupus (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). Therefore, considering clinical and laboratory criteria, she presented 4 or 11 classification criteria for SLE (positive ANA, painful, repeated oral ulcers, leukopenia, discoid lupus). She was treated with hydroxicloroquine with a reduction in skin lesions after approximately 2 weeks (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0015" class="elsevierStylePara elsevierViewall">EKF was described in 1972 and is characterized by the onset of fever and cervical lymphadenopathy.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a> It affects mainly young women. It is rare (found only in some series of cases<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a>), and was first described in Japan, but is becoming more common in western countries.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Its etiology is unknown, although the most accepted hypothesis is that the etiology is viral.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2,5</span></a> It may be associated with different connective tissue diseases, such as Still's disease, Sjögren syndrome, polymyositis, rheumatoid arthritis, and above all, SLE.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,6</span></a> Common symptoms at presentation are fever, myalgia, weight loss, nausea and vomiting as well as skin lesions.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3,7</span></a> The differential diagnosis includes infections (toxoplasmosis, mononucleosis, tuberculosis, etc.) and lymphoma.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3,5</span></a> Laboratory tests may show leukopenia, mild thrombocytopenia and neutropenia, and increased acute phase reactants, mainly ESR.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5,8</span></a> Histology reveals paracortical foci of necrosis with abundant nuclear debris, histiocyte infiltrates, with no neutrophils.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3,7,8</span></a> KHD and lupus lymphadenitis may be histologically identical. Only in a few cases where the pathologist can observe hematoxiphillic bodies (aggregates of basophilic material in the lymph node sinuses), accumulation of DNA in the vessel wall, vasculitis or areas outside the area of necrosis which suggest lupus lymphadenitis and KHD.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> The literature contains several case reports of KHD associated with SLE, and EKF may occur before, after or simultaneously with SLE.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5,7–12</span></a> The main difference between them is the prognosis, with the majority of cases of KHD resolving spontaneously in 1–4 months.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,8</span></a></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conclusions</span><p id="par0020" class="elsevierStylePara elsevierViewall">In this case, the patient was initially diagnosed with a histologically confirmed KHD in which we ruled out other possible causes, mainly infectious or hematologic disease, as the origin of lymphadenopathy and fever. We believe this case supports the recommendation that a possible association between KFD and SLE should be always considered, as well as the need to closely monitor the progression of KHD with SLE.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:5 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Case Description" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Conclusions" ] 4 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please cite this article as: Diez-Morrondo C, et al. Inicio de lupus eritematoso sistémico como enfermedad de Kikuchi-Fujimoto. Reumatol Clin. 2012;<span class="elsevierStyleBold">8</span>:153–5.</p>" ] ] "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 471 "Ancho" => 1252 "Tamanyo" => 272706 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Necrotizing lymphadenitis: zones of necrosis with cellular debris but no neutrophils, surrounded by lymphoid cells and hystiocytes (left). There is a zone of necrosis with numerous hystiocytes and large reactive lymphoid cells (right).</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 658 "Ancho" => 701 "Tamanyo" => 57508 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Initial skin manifestations.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 987 "Ancho" => 1301 "Tamanyo" => 433807 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Discoid lupus: periannex lymphoid dermatitis (upper left) with vacuolization of the basal epidermal zone (upper right). Abundant mucin deposits in the reticular dermis (lower left) which are more evident with a colloidal iron stain (lower right).</p>" ] ] 3 => array:7 [ "identificador" => "fig0020" "etiqueta" => "Fig. 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 689 "Ancho" => 700 "Tamanyo" => 41946 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Response to hydroxicloroquine.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:12 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Kikuchi–Fujimoto Disease: analysis of 244 cases" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "Y. Kucukardali" 1 => "E. Solmazgul" 2 => "E. Kunter" 3 => "O. Oncul" 4 => "S. Yildirim" 5 => "M. Kaplan" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s10067-006-0230-5" "Revista" => array:6 [ "tituloSerie" => "Clin Rheumatol" "fecha" => "2007" "volumen" => "26" "paginaInicial" => "50" "paginaFinal" => "54" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16538388" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Enfermedad de Kikuchi–Fujimoto: descripción de dos casos" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "A. Toledano Muñoz" 1 => "G. García de Casasola" 2 => "M. Argüelles Pintos" 3 => "G. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 16 | 10 | 26 |
| 2024 October | 91 | 66 | 157 |
| 2024 September | 110 | 46 | 156 |
| 2024 August | 149 | 59 | 208 |
| 2024 July | 120 | 59 | 179 |
| 2024 June | 96 | 38 | 134 |
| 2024 May | 124 | 33 | 157 |
| 2024 April | 97 | 31 | 128 |
| 2024 March | 81 | 38 | 119 |
| 2024 February | 73 | 36 | 109 |
| 2024 January | 67 | 29 | 96 |
| 2023 December | 58 | 31 | 89 |
| 2023 November | 56 | 40 | 96 |
| 2023 October | 80 | 28 | 108 |
| 2023 September | 121 | 56 | 177 |
| 2023 August | 78 | 26 | 104 |
| 2023 July | 69 | 29 | 98 |
| 2023 June | 81 | 27 | 108 |
| 2023 May | 64 | 23 | 87 |
| 2023 April | 77 | 16 | 93 |
| 2023 March | 159 | 36 | 195 |
| 2023 February | 113 | 39 | 152 |
| 2023 January | 76 | 19 | 95 |
| 2022 December | 80 | 37 | 117 |
| 2022 November | 81 | 25 | 106 |
| 2022 October | 94 | 38 | 132 |
| 2022 September | 62 | 32 | 94 |
| 2022 August | 84 | 50 | 134 |
| 2022 July | 68 | 35 | 103 |
| 2022 June | 59 | 38 | 97 |
| 2022 May | 63 | 44 | 107 |
| 2022 April | 54 | 50 | 104 |
| 2022 March | 67 | 45 | 112 |
| 2022 February | 84 | 37 | 121 |
| 2022 January | 73 | 33 | 106 |
| 2021 December | 33 | 40 | 73 |
| 2021 November | 46 | 49 | 95 |
| 2021 October | 60 | 50 | 110 |
| 2021 September | 54 | 52 | 106 |
| 2021 August | 38 | 49 | 87 |
| 2021 July | 44 | 35 | 79 |
| 2021 June | 52 | 41 | 93 |
| 2021 May | 53 | 40 | 93 |
| 2021 April | 113 | 81 | 194 |
| 2021 March | 92 | 40 | 132 |
| 2021 February | 46 | 31 | 77 |
| 2021 January | 46 | 29 | 75 |
| 2020 December | 54 | 23 | 77 |
| 2020 November | 35 | 13 | 48 |
| 2020 October | 24 | 13 | 37 |
| 2020 September | 40 | 24 | 64 |
| 2020 August | 24 | 17 | 41 |
| 2020 July | 30 | 28 | 58 |
| 2020 June | 41 | 30 | 71 |
| 2020 May | 37 | 20 | 57 |
| 2020 April | 23 | 12 | 35 |
| 2020 March | 22 | 1 | 23 |
| 2020 February | 1 | 0 | 1 |
| 2020 January | 4 | 0 | 4 |
| 2019 September | 6 | 0 | 6 |
| 2019 June | 2 | 0 | 2 |
| 2019 March | 2 | 0 | 2 |
| 2019 January | 1 | 0 | 1 |
| 2018 May | 5 | 0 | 5 |
| 2018 April | 90 | 10 | 100 |
| 2018 March | 89 | 14 | 103 |
| 2018 February | 41 | 7 | 48 |
| 2018 January | 36 | 4 | 40 |
| 2017 December | 47 | 7 | 54 |
| 2017 November | 38 | 9 | 47 |
| 2017 October | 48 | 4 | 52 |
| 2017 September | 59 | 8 | 67 |
| 2017 August | 46 | 3 | 49 |
| 2017 July | 41 | 10 | 51 |
| 2017 June | 47 | 19 | 66 |
| 2017 May | 72 | 8 | 80 |
| 2017 April | 39 | 6 | 45 |
| 2017 March | 40 | 3 | 43 |
| 2017 February | 65 | 7 | 72 |
| 2017 January | 39 | 6 | 45 |
| 2016 December | 87 | 12 | 99 |
| 2016 November | 98 | 12 | 110 |
| 2016 October | 123 | 14 | 137 |
| 2016 September | 225 | 7 | 232 |
| 2016 August | 86 | 5 | 91 |
| 2016 July | 61 | 4 | 65 |
| 2016 April | 1 | 0 | 1 |
| 2015 December | 2 | 0 | 2 |
| 2015 September | 1 | 0 | 1 |
| 2015 August | 2 | 0 | 2 |
| 2015 July | 43 | 6 | 49 |
| 2015 June | 57 | 13 | 70 |
| 2015 May | 90 | 19 | 109 |
| 2015 April | 70 | 12 | 82 |
| 2015 March | 58 | 3 | 61 |
| 2015 February | 66 | 17 | 83 |
| 2015 January | 89 | 14 | 103 |
| 2014 December | 74 | 10 | 84 |
| 2014 November | 62 | 20 | 82 |
| 2014 October | 88 | 12 | 100 |
| 2014 September | 65 | 12 | 77 |
| 2014 August | 81 | 13 | 94 |
| 2014 July | 69 | 18 | 87 |
| 2014 June | 101 | 14 | 115 |
| 2014 May | 75 | 22 | 97 |
| 2014 April | 75 | 15 | 90 |
| 2014 March | 95 | 17 | 112 |
| 2014 February | 84 | 17 | 101 |
| 2014 January | 61 | 14 | 75 |
| 2013 December | 55 | 23 | 78 |
| 2013 November | 57 | 17 | 74 |
| 2013 October | 68 | 16 | 84 |
| 2013 September | 64 | 14 | 78 |
| 2013 August | 57 | 18 | 75 |
| 2013 July | 58 | 18 | 76 |
| 2013 June | 52 | 16 | 68 |
| 2013 May | 51 | 18 | 69 |
| 2013 April | 48 | 16 | 64 |
| 2013 March | 46 | 22 | 68 |
| 2013 February | 47 | 15 | 62 |
| 2013 January | 48 | 21 | 69 |
| 2012 December | 50 | 14 | 64 |
| 2012 November | 25 | 26 | 51 |
| 2012 October | 48 | 34 | 82 |
| 2012 September | 28 | 3 | 31 |
| 2012 July | 4 | 0 | 4 |
| 2012 June | 7 | 0 | 7 |
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