was read the article
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It was published in 1974 by Liliane Schnitzler, a French dermatologist, as an autonomous entity.</p><p id="par0010" class="elsevierStylePara elsevierViewall">It is characterized as a rare, acquired syndrome, which shares common characteristics with a group of inherited diseases known as the autoinflammatory syndromes. It has been described most often in men between the fourth and fifth decades of life. Its etiology is unknown, but has been associated with conditions such as impaired balance of interleukin-1. The presenting symptoms are fever, rash and joint pain; there may also be lymphadenopathy and an IgM monoclonal component. 15%–20% of patients with this entity develop a lymphoproliferative syndrome. Conventional treatments are ineffective, and they include antihistamines, anti-inflammatory drugs, corticosteroids and immunosuppressants.</p><p id="par0015" class="elsevierStylePara elsevierViewall">We report the case of a 65-year-old male patient with no medical history, with a 10-year history consisting of intermittent fever, joint pain of moderate intensity, scattered maculopapular pruritic skin lesions located on the thorax, abdomen and lower extremities. Three months before our evaluation he developed back pain radiating to the left lower extremity, non-continuous but intense that prevented walking. The general examination showed the presence of a maculopapular rash on the chest, abdomen and extremities, as well as a positive Lassegue sign on the left leg.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Lab tests, including complete blood count, biochemistry, coagulation, serology, tumor markers, serum protein and immunoelectrophoresis, were normal, except for the presence of monoclonal IgM kappa and increased erythrocyte sedimentation rate (ESR).</p><p id="par0025" class="elsevierStylePara elsevierViewall">The skin lesion was biopsied. Histologic examination showed a superficial perivascular lymphocytic infiltration, interstitial dispersion and some neutrophils along collagen bundles.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> A cervical, thorax and abdomen computed tomography was performed, which showed no significant findings.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Given the different hematological findings, we performed aspiration/biopsy of bone marrow, which was consistent with Waldenstrom's disease. The patient is now in outpatient follow-up. No chemotherapy was started and has remained on symptomatic treatment only.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Schnitzler's syndrome is a rare entity which is difficult to diagnose given the absence of peripheral biomarkers and the need for confirmation of the diagnosis by a combination of clinical, laboratory and radiological tests as well as the exclusion of other diseases.</p><p id="par0040" class="elsevierStylePara elsevierViewall">To date, on the basis of criteria proposed by Lipsker et al. no more than 60 cases have been described. These criteria include the presence of an urticarial skin rash and a monoclonal IgM component and at least two of the following signs or symptoms: fever, joint pain or arthritis, bone pain, palpable lymph nodes, spleen, hepatomegaly, accelerated ESR and leukocytosis.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,3</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Our patient met criteria for Schnitzler syndrome, since some clinical (urticarial rash and fever) and laboratory findings (monoclonal IgM peak and increased ESR) present.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Among the various forms described above as part of the development of the disease, some authors include developing lymphoproliferative syndrome as lymphoplasmocytic lymphoma, Richter's syndrome, and<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> marginal lymphoma.</p><p id="par0055" class="elsevierStylePara elsevierViewall">The differential diagnosis of Schnitzler's syndrome should be performed with different entities, namely: cryoglobulinemia, hypocomplementemic urticarial vasculitis, acquired deficiency of C1 inhibitor, hyper IgD syndrome and adult Still's disease.</p><p id="par0060" class="elsevierStylePara elsevierViewall">The interest of this article is that the presence of monoclonal IgM, and the histology of skin lesions were alterations leading to the diagnosis of Schnitzler's syndrome with progression to Waldenstrom's disease. It should be stressed that a good history is essential, as it allows to know the exact nature of its evolution. Finally, we emphasize the importance of a differential diagnosis between<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Schnitzler's syndrome and Waldeströn's disease (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>) as well as the importance of knowing this entity to prevent the diagnosis from going unnoticed.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Herráez Albendea MM, et al. Síndrome de Schnitzler. Reumatol Clin. 2013;9:384.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "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=""><thead title="thead"><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" style="border-bottom: 2px solid black">Waldenström's disease \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" style="border-bottom: 2px solid black">Schnitzler's syndrome \t\t\t\t\t\t\n \t\t\t\t</td></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Monoclonal component \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Present \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Present \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Urticarial rash \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Absent \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Present \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Bone pain \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Absent/present \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Present/absent \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Hepato/splenomegaly \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Present/absent \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Present/absent \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Leukocytosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Absent \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Present/absent \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">IgM monoclonal protein \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Present \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Present \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Bone marrow infiltration \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Present \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Absent \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab427649.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Differences Between Schintzler's Syndrome and Waldeström's Disease.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clinical and 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Lipsker" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1186/1750-1172-5-38" "Revista" => array:5 [ "tituloSerie" => "Orphanet J Rare Dis" "fecha" => "2010" "volumen" => "5" "paginaInicial" => "38" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21143856" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Schnitzler syndrome, an autoimmune–autoinflammatory syndrome: report of two new cases and review of the literature" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "E. Tinazzi" 1 => "A. Puccetti" 2 => "G. Patuzzo" 3 => "M. Sorleto" 4 => "A. Barbieri" 5 => "C. Lunardi" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.autrev.2011.01.003" "Revista" => array:6 [ "tituloSerie" => "Autoimmun Rev" "fecha" => "2011" "volumen" => "10" "paginaInicial" => "404" "paginaFinal" => "409" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21256251" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Schnitzler's syndrome (urticaria and macroglobulinemia): evolution to Waldenström's disease is not uncommon" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "L. Machet" 1 => "L. Vaillant" 2 => "M.C. Machet" 3 => "M. Reisenleiter" 4 => "P. Goupille" 5 => "G. 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Year/Month | Html | Total | |
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2024 November | 10 | 14 | 24 |
2024 October | 84 | 37 | 121 |
2024 September | 81 | 15 | 96 |
2024 August | 100 | 32 | 132 |
2024 July | 130 | 28 | 158 |
2024 June | 121 | 31 | 152 |
2024 May | 134 | 34 | 168 |
2024 April | 83 | 22 | 105 |
2024 March | 107 | 33 | 140 |
2024 February | 84 | 24 | 108 |
2024 January | 93 | 23 | 116 |
2023 December | 104 | 26 | 130 |
2023 November | 93 | 27 | 120 |
2023 October | 87 | 31 | 118 |
2023 September | 100 | 39 | 139 |
2023 August | 63 | 11 | 74 |
2023 July | 55 | 30 | 85 |
2023 June | 68 | 18 | 86 |
2023 May | 71 | 23 | 94 |
2023 April | 32 | 13 | 45 |
2023 March | 50 | 24 | 74 |
2023 February | 50 | 32 | 82 |
2023 January | 32 | 13 | 45 |
2022 December | 33 | 34 | 67 |
2022 November | 64 | 26 | 90 |
2022 October | 49 | 28 | 77 |
2022 September | 49 | 30 | 79 |
2022 August | 41 | 46 | 87 |
2022 July | 35 | 42 | 77 |
2022 June | 34 | 30 | 64 |
2022 May | 36 | 30 | 66 |
2022 April | 34 | 42 | 76 |
2022 March | 35 | 35 | 70 |
2022 February | 32 | 24 | 56 |
2022 January | 22 | 28 | 50 |
2021 December | 33 | 41 | 74 |
2021 November | 43 | 39 | 82 |
2021 October | 33 | 61 | 94 |
2021 September | 29 | 34 | 63 |
2021 August | 27 | 40 | 67 |
2021 July | 28 | 42 | 70 |
2021 June | 39 | 35 | 74 |
2021 May | 27 | 22 | 49 |
2021 April | 64 | 43 | 107 |
2021 March | 67 | 25 | 92 |
2021 February | 46 | 23 | 69 |
2021 January | 47 | 22 | 69 |
2020 December | 31 | 30 | 61 |
2020 November | 51 | 17 | 68 |
2020 October | 22 | 7 | 29 |
2020 September | 28 | 20 | 48 |
2020 August | 22 | 15 | 37 |
2020 July | 27 | 24 | 51 |
2020 June | 27 | 25 | 52 |
2020 May | 28 | 15 | 43 |
2020 April | 26 | 24 | 50 |
2020 March | 19 | 6 | 25 |
2020 February | 1 | 0 | 1 |
2020 January | 2 | 0 | 2 |
2019 September | 2 | 0 | 2 |
2019 June | 1 | 0 | 1 |
2019 April | 2 | 0 | 2 |
2019 January | 1 | 0 | 1 |
2018 May | 5 | 1 | 6 |
2018 April | 34 | 6 | 40 |
2018 March | 84 | 10 | 94 |
2018 February | 25 | 3 | 28 |
2018 January | 21 | 6 | 27 |
2017 December | 32 | 4 | 36 |
2017 November | 26 | 7 | 33 |
2017 October | 26 | 3 | 29 |
2017 September | 36 | 3 | 39 |
2017 August | 44 | 4 | 48 |
2017 July | 45 | 14 | 59 |
2017 June | 60 | 4 | 64 |
2017 May | 53 | 11 | 64 |
2017 April | 29 | 4 | 33 |
2017 March | 44 | 5 | 49 |
2017 February | 17 | 5 | 22 |
2017 January | 32 | 4 | 36 |
2016 December | 65 | 13 | 78 |
2016 November | 47 | 2 | 49 |
2016 October | 62 | 16 | 78 |
2016 September | 73 | 11 | 84 |
2016 August | 71 | 7 | 78 |
2016 July | 39 | 7 | 46 |
2016 June | 0 | 12 | 12 |
2016 May | 0 | 9 | 9 |
2016 March | 0 | 15 | 15 |
2016 February | 0 | 19 | 19 |
2015 December | 2 | 0 | 2 |
2015 October | 1 | 15 | 16 |
2015 September | 2 | 0 | 2 |
2015 August | 1 | 0 | 1 |
2015 July | 32 | 9 | 41 |
2015 June | 41 | 2 | 43 |
2015 May | 47 | 13 | 60 |
2015 April | 34 | 7 | 41 |
2015 March | 33 | 4 | 37 |
2015 February | 49 | 11 | 60 |
2015 January | 32 | 12 | 44 |
2014 December | 33 | 10 | 43 |
2014 November | 30 | 11 | 41 |
2014 October | 39 | 12 | 51 |
2014 September | 29 | 14 | 43 |
2014 August | 40 | 19 | 59 |
2014 July | 27 | 15 | 42 |
2014 June | 46 | 18 | 64 |
2014 May | 50 | 20 | 70 |
2014 April | 51 | 14 | 65 |
2014 March | 72 | 24 | 96 |
2014 February | 50 | 13 | 63 |
2014 January | 70 | 25 | 95 |
2013 December | 50 | 35 | 85 |
2013 November | 24 | 6 | 30 |