Case for Diagnosis
Fever, Cervical Lymphadenopathy, and Erythematous-Violaceous Facial Plaques
Fiebre, linfadenopatías cervicales y placas eritematovioláceas faciales
A. Fueyo-Casado
, L. Campos-Muñoz, E. López-Bran
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Servicio de Dermatología, Hospital Clínico San Carlos, Madrid, Spain
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They measured several centimeters in diameter and were not tender (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Punch biopsy of a lesion showed a superficial and deep perivascular lymphocytic infiltrate, with slight vacuolization of the basal layer of the epidermis (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Laboratory tests including complete blood count, biochemistry, and coagulation studies gave normal results. The erythrocyte sedimentation rate was 30<span class="elsevierStyleHsp" style=""></span>mm. Antinuclear antibodies, rheumatoid factor, and serological tests for cytomegalovirus, herpes simplex virus types 1 and 2, hepatitis B and C viruses, human immunodeficiency virus (HIV), <span class="elsevierStyleItalic">Toxoplasma gondii, Bartonella henselae, Salmonella typhi, Brucella, Leptospira,</span> and <span class="elsevierStyleItalic">Francisella tularensis</span> were negative. Serological tests were positive for Epstein-Barr virus (EBV) and parvovirus B19 immunoglobulin [Ig] G. The Mantoux test and chest radiograph were negative.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Ultrasound of the cervical region confirmed lymphadenopathies. Biopsy of a node revealed paracortical areas of apoptotic cell death without abscess formation, abundant karyorrhexis, and a proliferation of histiocytes and plasmacytoid monocytes, with an absence of neutrophils (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>).</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis?</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Diagnosis</span><p id="par0035" class="elsevierStylePara elsevierViewall">Kikuchi-Fujimoto disease with skin involvement.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Clinical Course</span><p id="par0040" class="elsevierStylePara elsevierViewall">Treatment was started with low-dose oral and topical corticosteroids. After 8 weeks, the fever and lymphadenopathies had resolved. However, the skin lesions persisted, and treatment was therefore started with hydroxychloroquine (400<span class="elsevierStyleHsp" style=""></span>mg/d). There have been no repeat episodes of fever or lymphadenopathies, and only residual skin lesions remain after 2 years’ treatment.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0045" class="elsevierStylePara elsevierViewall">First described in 1972,<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare condition, which invariably resolves in a few weeks.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> The etiology is uncertain, although apoptosis plays a significant role. It could represent an exaggerated T-cell mediated response to certain antigens in genetically predisposed individuals. It has been suggested that Kikuchi-Fujimoto disease is triggered by autoimmune disease or infection by certain viruses such as Epstein-Barr virus, human herpesvirus 6, human herpesvirus 8, HIV, herpes simplex virus, hepatitis B virus, human T-cell leukemia virus type 1, parvovirus B19, paramyxovirus, and parainfluenza virus, in addition to other microorganisms such as <span class="elsevierStyleItalic">Yersinia enterocolitica</span> and <span class="elsevierStyleItalic">Toxoplasma</span>. It has also been reported with Still disease, antiphospholipid syndrome, hemophagocytic syndrome in children, brachial plexus neuritis, mononeuritis multiplex, bilateral panuveitis, aseptic meningitis, brucellosis, and Wells syndrome. Kikuchi-Fujimoto disease mainly affects young women and is clinically identified by painful cervical lymphadenopathy associated with fever, myalgia, neutropenia, and rarely (less than 40% of cases) skin rash.</p><p id="par0050" class="elsevierStylePara elsevierViewall">From a dermatological perspective, lesions have been described as macules, papules, plaques, nodules, and ulcers, on the upper part of the body (face, arms, and trunk).<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,4</span></a> Skin biopsy usually shows a superficial and deep perivascular infiltrate, edema of the papillary dermis, vacuolization, and necrotic keratinocytes in the basal layer of the epidermis. Patchy infiltrates of lymphoid cells and histiocytes and nuclear remnants similar to those found in the lymph node are also typical.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Diagnosis requires histopathological study of a lymph node biopsy and the differential diagnosis must be made with Hodgkin disease, high-grade lymphoma, and lymphadenitis associated with systemic lupus erythematosus. Infectious lymphadenitis (EBV, herpes virus, <span class="elsevierStyleItalic">Bartonella henselae</span>, or <span class="elsevierStyleItalic">Toxoplasma</span>) should also be excluded.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Treatment requires supportive measures and occasionally the administration of low-dose oral corticosteroids. Although the disease is self-limiting and resolves in a few weeks, skin manifestations are more persistent and must be managed in a similar way to those of cutaneous lupus erythematosus.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflicts of Interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 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" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0040" "titulo" => "Conflicts of Interest" ] 8 => array:2 [ "identificador" => "xack35191" "titulo" => "Acknowledgements" ] 9 => 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: Fueyo-Casado A, et al. Fiebre, linfadenopatías cervicales y placas eritematovioláceas faciales. Actas Dermosifiliogr. 2011;102:629-30.</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" => 508 "Ancho" => 675 "Tamanyo" => 91404 ] ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 658 "Ancho" => 510 "Tamanyo" => 117046 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin-eosin, original magnification x4.</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" => 537 "Ancho" => 715 "Tamanyo" => 216081 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin-eosin, original magnification x20.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytosis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "M. 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Kobayashi" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:7 [ "tituloSerie" => "Br J Dermatol" "fecha" => "2001" "volumen" => "144" "paginaInicial" => "885" "paginaFinal" => "889" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/11298555" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S0002939408009483" "estado" => "S300" "issn" => "00029394" ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Kikuchi-Fujimoto disease with papulopustular skin manifestations" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "K.J. Kim" 1 => "M.S. Jee" 2 => "S.E. Chang" 3 => "J.H. Choi" 4 => "K.J. Sung" 5 => "K.C. Moon" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Clin Exp Dermatol" "fecha" => "2003" "volumen" => "28" "paginaInicial" => "142" "paginaFinal" => "144" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12653698" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The histopathology of cutaneous lesions of Kikuchi's disease (necrotizing lymphadenitis): a report of five cases" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "J. Spies" 1 => "K. Foucar" 2 => "C.T. Thompson" 3 => "P.E. LeBoit" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Am J Surg Pathol" "fecha" => "1999" "volumen" => "23" "paginaInicial" => "1040" "paginaFinal" => "1047" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/10478663" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] "agradecimientos" => array:1 [ 0 => array:3 [ "identificador" => "xack35191" "titulo" => "Acknowledgements" "texto" => "<p id="par0070" class="elsevierStylePara elsevierViewall">We are grateful to our pathology department, without whose help it would not be possible to establish many diagnoses.</p>" ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000010200000008/v1_201304241234/S1578219011000679/v1_201304241234/en/main.assets" "Apartado" => array:4 [ "identificador" => "6154" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Case for Diagnosis" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15782190/0000010200000008/v1_201304241234/S1578219011000679/v1_201304241234/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219011000679?idApp=UINPBA000044" ]
| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 16 | 10 | 26 |
| 2024 Octubre | 106 | 45 | 151 |
| 2024 Septiembre | 133 | 38 | 171 |
| 2024 Agosto | 181 | 56 | 237 |
| 2024 Julio | 153 | 48 | 201 |
| 2024 Junio | 149 | 30 | 179 |
| 2024 Mayo | 116 | 31 | 147 |
| 2024 Abril | 123 | 22 | 145 |
| 2024 Marzo | 132 | 34 | 166 |
| 2024 Febrero | 107 | 28 | 135 |
| 2024 Enero | 98 | 32 | 130 |
| 2023 Diciembre | 113 | 18 | 131 |
| 2023 Noviembre | 104 | 30 | 134 |
| 2023 Octubre | 105 | 20 | 125 |
| 2023 Septiembre | 91 | 29 | 120 |
| 2023 Agosto | 99 | 15 | 114 |
| 2023 Julio | 130 | 48 | 178 |
| 2023 Junio | 111 | 18 | 129 |
| 2023 Mayo | 137 | 27 | 164 |
| 2023 Abril | 84 | 18 | 102 |
| 2023 Marzo | 79 | 27 | 106 |
| 2023 Febrero | 128 | 17 | 145 |
| 2023 Enero | 91 | 27 | 118 |
| 2022 Diciembre | 69 | 45 | 114 |
| 2022 Noviembre | 58 | 25 | 83 |
| 2022 Octubre | 59 | 20 | 79 |
| 2022 Septiembre | 53 | 46 | 99 |
| 2022 Agosto | 49 | 28 | 77 |
| 2022 Julio | 53 | 35 | 88 |
| 2022 Junio | 44 | 30 | 74 |
| 2022 Mayo | 96 | 31 | 127 |
| 2022 Abril | 110 | 35 | 145 |
| 2022 Marzo | 145 | 43 | 188 |
| 2022 Febrero | 147 | 24 | 171 |
| 2022 Enero | 120 | 34 | 154 |
| 2021 Diciembre | 85 | 45 | 130 |
| 2021 Noviembre | 72 | 44 | 116 |
| 2021 Octubre | 76 | 48 | 124 |
| 2021 Septiembre | 59 | 38 | 97 |
| 2021 Agosto | 61 | 36 | 97 |
| 2021 Julio | 54 | 22 | 76 |
| 2021 Junio | 69 | 37 | 106 |
| 2021 Mayo | 64 | 36 | 100 |
| 2021 Abril | 133 | 39 | 172 |
| 2021 Marzo | 87 | 25 | 112 |
| 2021 Febrero | 86 | 31 | 117 |
| 2021 Enero | 64 | 14 | 78 |
| 2020 Diciembre | 57 | 11 | 68 |
| 2020 Noviembre | 42 | 24 | 66 |
| 2020 Octubre | 27 | 7 | 34 |
| 2020 Septiembre | 32 | 7 | 39 |
| 2020 Agosto | 30 | 17 | 47 |
| 2020 Julio | 30 | 15 | 45 |
| 2020 Junio | 44 | 17 | 61 |
| 2020 Mayo | 35 | 18 | 53 |
| 2020 Abril | 34 | 20 | 54 |
| 2020 Marzo | 34 | 32 | 66 |
| 2020 Febrero | 3 | 5 | 8 |
| 2020 Enero | 0 | 5 | 5 |
| 2019 Diciembre | 4 | 2 | 6 |
| 2019 Octubre | 0 | 1 | 1 |
| 2019 Septiembre | 6 | 2 | 8 |
| 2019 Agosto | 0 | 6 | 6 |
| 2019 Julio | 0 | 2 | 2 |
| 2019 Junio | 0 | 17 | 17 |
| 2019 Mayo | 2 | 13 | 15 |
| 2019 Abril | 0 | 7 | 7 |
| 2019 Marzo | 0 | 9 | 9 |
| 2019 Febrero | 2 | 2 | 4 |
| 2018 Diciembre | 0 | 5 | 5 |
| 2018 Noviembre | 2 | 0 | 2 |
| 2018 Octubre | 1 | 0 | 1 |
| 2018 Septiembre | 2 | 0 | 2 |
| 2018 Mayo | 0 | 4 | 4 |
| 2018 Marzo | 4 | 0 | 4 |
| 2018 Febrero | 46 | 5 | 51 |
| 2018 Enero | 57 | 9 | 66 |
| 2017 Diciembre | 60 | 7 | 67 |
| 2017 Noviembre | 50 | 6 | 56 |
| 2017 Octubre | 61 | 7 | 68 |
| 2017 Septiembre | 58 | 7 | 65 |
| 2017 Agosto | 80 | 8 | 88 |
| 2017 Julio | 45 | 8 | 53 |
| 2017 Junio | 73 | 20 | 93 |
| 2017 Mayo | 54 | 14 | 68 |
| 2017 Abril | 59 | 10 | 69 |
| 2017 Marzo | 53 | 31 | 84 |
| 2017 Febrero | 39 | 11 | 50 |
| 2017 Enero | 26 | 12 | 38 |
| 2016 Diciembre | 62 | 6 | 68 |
| 2016 Noviembre | 60 | 7 | 67 |
| 2016 Octubre | 82 | 11 | 93 |
| 2016 Septiembre | 66 | 13 | 79 |
| 2016 Agosto | 51 | 6 | 57 |
| 2016 Julio | 33 | 1 | 34 |
| 2016 Junio | 9 | 5 | 14 |
| 2016 Mayo | 7 | 6 | 13 |
| 2016 Abril | 4 | 1 | 5 |
| 2016 Marzo | 7 | 3 | 10 |
| 2016 Febrero | 8 | 3 | 11 |
| 2016 Enero | 4 | 0 | 4 |
| 2015 Diciembre | 7 | 3 | 10 |
| 2015 Noviembre | 8 | 1 | 9 |
| 2015 Octubre | 6 | 1 | 7 |
| 2015 Septiembre | 6 | 3 | 9 |
| 2015 Agosto | 14 | 4 | 18 |
| 2015 Julio | 66 | 4 | 70 |
| 2015 Junio | 44 | 7 | 51 |
| 2015 Mayo | 82 | 14 | 96 |
| 2015 Abril | 43 | 10 | 53 |
| 2015 Marzo | 66 | 5 | 71 |
| 2015 Febrero | 50 | 6 | 56 |
| 2015 Enero | 57 | 13 | 70 |
| 2014 Diciembre | 64 | 12 | 76 |
| 2014 Noviembre | 45 | 17 | 62 |
| 2014 Octubre | 52 | 16 | 68 |
| 2014 Septiembre | 39 | 9 | 48 |
| 2014 Agosto | 49 | 11 | 60 |
| 2014 Julio | 52 | 15 | 67 |
| 2014 Junio | 71 | 12 | 83 |
| 2014 Mayo | 87 | 15 | 102 |
| 2014 Abril | 50 | 13 | 63 |
| 2014 Marzo | 68 | 17 | 85 |
| 2014 Febrero | 49 | 10 | 59 |
| 2014 Enero | 52 | 10 | 62 |
| 2013 Diciembre | 58 | 10 | 68 |
| 2013 Noviembre | 44 | 19 | 63 |
| 2013 Octubre | 61 | 14 | 75 |
| 2013 Septiembre | 12 | 3 | 15 |
| 2013 Agosto | 16 | 12 | 28 |
| 2013 Julio | 12 | 13 | 25 |
| 2013 Junio | 10 | 13 | 23 |
| 2013 Mayo | 16 | 3 | 19 |
| 2013 Abril | 9 | 17 | 26 |
| 2013 Marzo | 11 | 8 | 19 |
| 2013 Febrero | 30 | 5 | 35 |
| 2013 Enero | 29 | 3 | 32 |
| 2012 Diciembre | 18 | 4 | 22 |
| 2012 Noviembre | 1 | 0 | 1 |
| 2012 Octubre | 0 | 2 | 2 |
| 2012 Agosto | 0 | 1 | 1 |
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