Case and Research Letter
Familial Mediterranean Fever: Diagnostic Difficulties in an Atypical Case
Fiebre mediterránea familiar. Dificultades diagnósticas en un caso atípico
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A, Nodule in the mesogastric region. B, Color Doppler, longitudinal axis, 18<span class="elsevierStyleHsp" style=""></span>Mhz. In the subcutaneous cellular tissue, a well-defined tumor formed of anechoic lacunae surrounded by hypoechoic septa with flow in their interior (center and left of the image) and destructured hypoechoic areas (right side of the image). C, Macroscopic appearance of the tumor after surgical excision, showing the lacunar structures visible on ultrasound. D, Ectasias containing red blood cells but with no endothelium, surrounded by cells of fibrohistiocytic appearance. Hematoxylin and eosin, original magnification<span class="elsevierStyleHsp" style=""></span>x100.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "B. Echeverría-García, C. García-Donoso, J.C. Tardío, J. Borbujo" "autores" => array:4 [ 0 => array:2 [ "nombre" => "B." "apellidos" => "Echeverría-García" ] 1 => array:2 [ "nombre" => "C." 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Loidi Pascual, M. Larrea García, C. Llanos Chávarri, J.I. Yanguas Bayona" "autores" => array:4 [ 0 => array:4 [ "nombre" => "L." "apellidos" => "Loidi Pascual" "email" => array:1 [ 0 => "leyre.loidi.pascual@cfnavarra.es" ] "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" => "M." "apellidos" => "Larrea García" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "C." "apellidos" => "Llanos Chávarri" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "J.I." "apellidos" => "Yanguas Bayona" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Complejo Hospitalario de Navarra, Pamplona, Navarra, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Complejo Hospitalario de Navarra, Pamplona, Navarra, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Fiebre mediterránea familiar. Dificultades diagnósticas en un caso atípico" ] ] "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" => 1164 "Ancho" => 1650 "Tamanyo" => 458565 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Edema associated with a moderate inflammatory infiltrate of lymphocytes and neutrophils in the superficial dermis, with occasional images of leukocytoclasia. No extravasation of red blood cells is observed, nor the presence of hemosiderophages or lesions of fibrinoid necrosis in the vessel walls. Hematoxylin and eosin, original magnification<span class="elsevierStyleHsp" style=""></span>×20.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 68-year-old woman with a history of neurosensorial deafness and anemia of chronic disease consulted for more than 20 years of recurrent episodes of fever, joint and muscle pain, and skin lesions on the arms and trunk. The episodes occurred every 2 to 6 months and lasted around 2 weeks. She was asymptomatic between episodes. Large edematous erythematous plaques arose on her trunk and the root of her upper limbs (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>), associated with fever of 38<span class="elsevierStyleHsp" style=""></span>°C. Blood tests were normal. Skin biopsy showed edema and an inflammatory infiltrate of lymphocytes and neutrophils in the superficial dermis, without fibrinoid necrosis (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">She subsequently developed fever associated with erythema and increased temperature in 1 of her legs, with blisters and an abundant exudate (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). A gram-negative microorganism was isolated on culture, and this was interpreted as infectious cellulitis. Four months later she presented similar manifestations in her other leg.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Genetic analysis was performed of genes <span class="elsevierStyleItalic">MEFV</span>, <span class="elsevierStyleItalic">TNFRSF1A</span>, <span class="elsevierStyleItalic">MVK</span>, <span class="elsevierStyleItalic">NLRP3</span>, <span class="elsevierStyleItalic">NOD2</span>, and <span class="elsevierStyleItalic">PSTPIP1</span>. The patient was heterozygous for the c.1772T>C variant of gene <span class="elsevierStyleItalic">MEFV</span>.</p><p id="par0020" class="elsevierStylePara elsevierViewall">As she also satisfied the Tel-Hashomer diagnostic criteria for familial Mediterranean fever (FMF) (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>), she was diagnosed with this entity. Treatment was started with colchicine, which led to a marked improvement in the clinical manifestations.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">FMF is the most common autoinflammatory disease of adults.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> It has a monogenic autosomal recessive inheritance, caused by a mutation in gene <span class="elsevierStyleItalic">MEFV</span> (16p13.3), although 20% of patients are heterozygous.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> This could be because it is actually an autosomal dominant disease with variable penetrance, it was not possible to detect the second mutation due to technical limitations, or that it could be a polygenic disease. Some authors have stated that these patients have a later onset of the disease, with a shorter duration of outbreaks, milder symptoms, and longer symptom-free intervals.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Gene <span class="elsevierStyleItalic">MEFV</span> codes the protein pyrin, also known as marenostrin, which plays a role in regulation of the innate immune response. Alteration of this protein removes control from the pathway, increasing the levels of interleukin 1β, responsible for the inflammatory response in this disease.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Soriano and Manna<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> described 4 clinical phenotypes of the disease:<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">–</span><p id="par0040" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Type 1:</span> Patients with recurrent episodes of short duration (12-72<span class="elsevierStyleHsp" style=""></span>h) with fever, acute abdominal pain, joint involvement, acute chest pain due to pleuritis/pericarditis, and various skin manifestations. Patients are usually asymptomatic between outbreaks, although biochemical evidence of the disease can be detected.</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">–</span><p id="par0045" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Type 2:</span> Patients who develop secondary amyloidosis (AA type) with proteinuria or kidney failure before they develop other signs of FMF, or as the only manifestation in relatives of patients with FMF.</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">–</span><p id="par0050" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Type 3:</span> Carriers of a mutation of gene <span class="elsevierStyleItalic">MEFV</span> with no clinical manifestations of the disease and no amyloidosis. This occurs in endemic populations (such as Iraqi Jews and Ashkenazi Jews), with a prevalence of 1 in 25 to 1 in 300 persons. Although the majority never present clinical manifestations, some do develop amyloidosis with time (phenotype<span class="elsevierStyleHsp" style=""></span>2).</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">–</span><p id="par0055" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">FMF-like:</span> Heterozygous patients with mild clinical manifestations of the disease. Our patient falls into this phenotype.</p></li></ul></p><p id="par0060" class="elsevierStylePara elsevierViewall">Cutaneous manifestations of the disease occur in 10% to 40% of affected patients.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> Only erysipelas- or cellulitis-like lesions, seen in 5% to 30% of patients with FMF, are considered specific, and they arise on the anterior surface of the legs and dorsum of the feet. Histology reveals a perivascular dermal infiltrate made up mainly of mononuclear cells and neutrophils, but with no vasculitis.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> The episodes that affected our patient's legs probably corresponded to this clinical presentation. Panniculitis can present as erythema nodosum or neutrophilic panniculitis. Urticarial manifestations are more typical of other autoinflammatory syndromes, such as TRAPS (tumor necrosis factor receptor associated periodic fever syndrome) and CAPS (cryopyrin-associated autoinflammatory syndromes).<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">The differential diagnosis should include other autoinflammatory diseases.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> In our case, we considered Muckle-Wells syndrome because of the urticarial lesions and neurosensorial deafness, although the genetic analysis finally confirmed the diagnosis of FMF. Some authors believe that patients with FMF may have a greater prevalence of auditory loss than the unaffected population, but there has been no evidence of this in any controlled study.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">The diagnostic criteria of FMF were established in 1997 (Table<span class="elsevierStyleHsp" style=""></span>1).<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> The diagnosis is clinical; genetic analysis is only used for support. In our opinion, genetic analysis may be fundamental to reaching the diagnosis in some cases. However, detection of a mutation cannot alone confirm the diagnosis, particularly in endemic populations in which prevalence of the mutation is very high.</p><p id="par0075" class="elsevierStylePara elsevierViewall">The main objectives of treatment are the prevention of outbreaks and, in the long-term, of secondary amyloidosis. The drug of choice is colchicine, 1-3<span class="elsevierStyleHsp" style=""></span>mg/d.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">In conclusion, in patients with recurrent episodes of fever with cutaneous manifestations, we should consider the autoinflammatory syndromes, which include FMF. The diagnosis is basically clinical, although genetic analysis can be very useful. This disease is classically considered to have autosomal recessive inheritance, although a notable percentage of heterozygous patients have symptoms, meaning that aspects of the genetics of this disease still remain to be clarified.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0020">Please cite this article as: Pascual LL, García ML, Chávarri CL, Bayona JIY. Fiebre mediterránea familiar. Dificultades diagnósticas en un caso atípico. Actas Dermosifiliogr. 2017;108:161–164.</p>" ] ] "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 724 "Ancho" => 2009 "Tamanyo" => 156593 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A and B, Large edematous erythematous plaques with an urticarial appearance and increased local temperature. The lesions were found on the trunk and root of the upper limbs.</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" => 1164 "Ancho" => 1650 "Tamanyo" => 458565 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Edema associated with a moderate inflammatory infiltrate of lymphocytes and neutrophils in the superficial dermis, with occasional images of leukocytoclasia. No extravasation of red blood cells is observed, nor the presence of hemosiderophages or lesions of fibrinoid necrosis in the vessel walls. Hematoxylin and eosin, original magnification<span class="elsevierStyleHsp" style=""></span>×20.</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" => 805 "Ancho" => 1247 "Tamanyo" => 182284 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Hot erythematous plaque on a leg, with blister formation and an abundant exudate.</p>" ] ] 3 => 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:2 [ "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"><th class="td-with-role" title="table-head ; entry_with_role_rowhead " align="left" valign="top" scope="col">Major Criteria \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col">Minor Criteria \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col">Data That Support the Diagnosis \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Typical episodes: recurrent, <72<span class="elsevierStyleHsp" style=""></span>hours, febrile<br><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>1. Diffuse peritonitis<br><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>2. Unilateral pleuritis or pericarditis<br><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>3. Monoarthritis of the lower limbs<br><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>4. Fever as the only manifestation \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top"><span class="elsevierStyleBold">Atypical episodes</span><a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">b</span></a>: no fever, longer duration, other manifestations different from 1-3 of the Major Criteria<br>4. Pain in the lower limbs on exertion<br>5. <span class="elsevierStyleBold">Favorable response to colchicine</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1. Family history of FMF<br>2. Typical ethnic origin<br>3. Onset<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>20 years<br>Characteristics of the episodes:<br>4. <span class="elsevierStyleBold">Marked alteration of the general state, requiring rest</span><br>5. <span class="elsevierStyleBold">Spontaneous remission</span><br>6. <span class="elsevierStyleBold">Symptom-free between episodes</span><br>7. Elevation of at least 1 AFR: fibrinogen, white cell count, ESR<br>8. Episodes of proteinuria or hematuria<br>9. Acute abdomen with negative exploratory laparotomy<br>10. Consanguinity \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1352938.png" ] ] ] "notaPie" => array:2 [ 0 => array:3 [ "identificador" => "tblfn0005" "etiqueta" => "a" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Diagnostic requirements: 1 mayor criterion, 2 minor criteria, 1 minor criterion<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>5 findings that support the diagnosis, or 4 of the first 5 parameters that support the diagnosis.</p>" ] 1 => array:3 [ "identificador" => "tblfn0010" "etiqueta" => "b" "nota" => "<p class="elsevierStyleNotepara" id="npar0010">The criteria in our patient, who satisfied 2 minor criteria and 4 findings that supported the diagnosis, are shown in bold type.</p> <p class="elsevierStyleNotepara" id="npar0015">AFR indicates acute phase reactant; ESR, erythrocyte sedimentation rate.</p>" ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Tel-Hashomer Diagnostic Criteria<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a></p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clinical and genetic characterization of the autoinflammatory diseases diagnosed in an adult reference center" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J. 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| año/Mes | Html | Total | |
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| 2021 Agosto | 58 | 38 | 96 |
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| 2019 Septiembre | 4 | 0 | 4 |
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| 2019 Febrero | 1 | 0 | 1 |
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| 2018 Noviembre | 2 | 0 | 2 |
| 2018 Octubre | 8 | 0 | 8 |
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| 2018 Febrero | 84 | 3 | 87 |
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| 2017 Diciembre | 161 | 5 | 166 |
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| 2017 Octubre | 24 | 4 | 28 |
| 2017 Septiembre | 20 | 11 | 31 |
| 2017 Agosto | 30 | 9 | 39 |
| 2017 Julio | 28 | 7 | 35 |
| 2017 Junio | 28 | 11 | 39 |
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