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It is classically defined as a series of changes in the color of the fingers and/or toes which occur in three distinct phases: first a phase of pallor (white), followed by a cyanotic phase (blue) and, finally, hyperemia (red).</p><p id="par0010" class="elsevierStylePara elsevierViewall">Not all the patients experience these three phases; in most cases, there is a single phase in which the color of the skin of the fingers or toes changes to a typically bluish color known as acrocyanosis, a circumstance that makes diagnosis more difficult. In contrast to Raynaud's phenomenon, acrocyanosis is a relatively common finding in infants and small children, and consists of bilateral, symmetrical discoloration of the hands and feet; it is a benign and self-limiting process that does not require treatment. Only in rare cases is acrocyanosis in children caused by Raynaud's phenomenon and, when this occurs, it needs immediate medical attention in order to prevent complications.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case Report</span><p id="par0015" class="elsevierStylePara elsevierViewall">We present the case of a boy aged 4 years and 10 months who was brought to his health center after having had episodes of cold, pallor and pain in both feet.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The family history included a 43-year-old father who had undergone removal of a benign gallbladder polyp, a healthy 42-year-old mother, 4 healthy older siblings, and paternal grandparents diagnosed with type 2 diabetes mellitus. The patient had been born in an vaginal delivery, after a normal, monitored pregnancy. His ponderal index at birth was in the 75th–90th percentile. He had no known allergies. At the age of 2 months, he had been hospitalized for acute otitis media. He was up to date with the immunization schedule of his Spanish autonomous community.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The patient had been brought to his health center after having experienced several episodes of pallor and a sensation of cold, followed by flushing, in both feet up to his ankles, which was accompanied by intense pain lasting approximately 30<span class="elsevierStyleHsp" style=""></span>min. The episodes were not related to abrupt changes in temperature, and the signs disappeared progressively with massage and local warming (<a class="elsevierStyleCrossRefs" href="#fig0005">Figs. 1 and 2</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Physical examination during that visit revealed no evidence of discoloration, nor were any anomalies found on palpation of symmetrical distal pulses.</p><p id="par0035" class="elsevierStylePara elsevierViewall">As these clinical signs led us to suspect Raynaud's phenomenon, we referred the patient to pediatric rheumatology, where blood tests, including liver and kidney function, and microbiological and autoantibody studies were found to be normal.</p><p id="par0040" class="elsevierStylePara elsevierViewall">In the vascular surgery unit, he underwent a Doppler ultrasound study of digital forward flow, which was normal. On qualitative capillaroscopy, there was no evidence of dilation, tortuosity or branching, and capillary function was normal; quantitative assessment of capillaries by capillaroscopy was also normal.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Given that the results of all the studies were normal, the patient was discharged from the hospital. At the present time, the episodes are more sporadic. They continue to subside with massage and local warming, and have no impact on the child's daily activities.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0050" class="elsevierStylePara elsevierViewall">The diagnosis in the case described in this article was Raynaud's phenomenon, based on the clinical course, the results of supplemental tests and the exclusion of other causes.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Raynaud's phenomenon is caused by vasospasm of the small arteries and arterioles of the fingers and toes. It is triggered by a number of stimuli, including exposure to cold or emotional stress. It can be symmetrical or involve an extremity on only one side. It is attributed mainly to hyperactivity of the sympathetic nervous system, together with an imbalance between vasodilator and vasoconstrictor substances. A number of studies have reported that the cutaneous nerve endings of patients diagnosed with Raynaud's phenomenon show an inadequate release of a potent vasodilator, the calcitonin-gene related peptide. This primary disorder may become more pronounced when associated with other factors, such as cold or emotional stress, which would trigger a cascade of compounds involved in vasoconstriction, such as catecholamines, endothelin-1, and 5-hydroxytryptamine. These mediators could be the ultimate cause of the endothelial damage seen in more severe cases.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Some studies have suggested that high blood levels of homocysteine may be associated with Raynaud's phenomenon because of its role, as an atherosclerotic factor, in reducing vasodilation.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Raynaud's phenomenon appears to have a strong familial component, which has led to the undertaking of genetic studies that have not yet been concluded. It is classified as:<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">–</span><p id="par0070" class="elsevierStylePara elsevierViewall">Secondary Raynaud's phenomenon: Normally, there is a well-defined primary disease, such as a connective tissue disease (systemic lupus erythematosus, polyarteritis nodosa or scleroderma), repeated trauma (work involving vibratory tools), drugs (interferon α, bleomycin), infections, metabolic diseases (cryoglobulinemia, atherosclerosis), exposure to chemical agents (tobacco, plastics), CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia), etc. It is associated with a high morbidity rate, and can even produce gangrene in the fingers or toes. It requires chronic treatment and the results of ancillary tests usually show alterations, including autoantibody positivity and anomalies observed in capillaroscopy, which reveals giant capillaries with irregular architecture and, in some cases, an avascular area.</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">–</span><p id="par0075" class="elsevierStylePara elsevierViewall">Primary Raynaud's phenomenon (previously referred to as Raynaud's disease): It presents in the absence of other diseases and the course is usually benign. The findings on capillaroscopy are normal and tests for antinuclear antibodies (ANA) are negative, features that differentiate it from secondary Raynaud's phenomenon.</p></li></ul></p><p id="par0080" class="elsevierStylePara elsevierViewall">Raynaud's phenomenon is extremely rare in children; the first report of this disease in children was in 1967, almost exactly 100 years after the initial description, in 1862. In general, according to published series, girls are more predisposed to develop Raynaud's phenomenon and the onset occurs during the changes observed around menarche, due to the influence of hormones.</p><p id="par0085" class="elsevierStylePara elsevierViewall">The largest cohort studied, published in 2003, involving 123 children with Raynaud's phenomenon, demonstrated that there is marked heterogeneity in terms of patient age, disease presentation and symptoms; for example, exposure to cold was the major trigger in the majority of the cases (roughly 70%) but, in 10%, no trigger was identified.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Primary Raynaud's phenomenon had a bimodal presentation, affecting young children and adolescents, and a considerable number of children, the majority under 2 years of age, were initially diagnosed as having acrocyanosis. The clues that should lead us to suspect Raynaud's phenomenon, rather than acrocyanosis, include the presence of atypical features, like a duration of more than 72<span class="elsevierStyleHsp" style=""></span>h and/or unilateral cyanosis.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2,3</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">Management of this disease is generally supportive and is based on an accurate diagnosis. Mild forms can be controlled by hygienic measures, such as the avoidance of exposure to cold or stressful situations, with no need for drug therapy. Moderate to severe cases require vasodilator therapy, such as systemic or topical calcium channel blockers. In very severe cases, intravenous infusion of prostacyclin, endothelin-1 receptor antagonists, inhibitors of phosphodiesterase-5, antiplatelet agents and antithrombotic therapy are being used with variable results. Surgery is reserved for extreme cases in which there is no response to the different drug therapies, and consists of digital sympathectomy.</p><p id="par0095" class="elsevierStylePara elsevierViewall">In recent years, new treatments are being investigated, including selective alpha-2c adrenergic receptor blockers, protein kinase inhibitors and calcitonin gene-related peptide.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">4,5</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">In patients with secondary Raynaud's phenomenon, specific treatment of the underlying disease is critical for controlling the episodes.</p><p id="par0105" class="elsevierStylePara elsevierViewall">Our patient appears to have primary Raynaud's phenomenon in its mildest form, as indicated by the clinical findings and the results of the tests performed. As we mentioned above, the treatment is supportive and consists in avoiding exposure to intense cold and situations of major stress. We think it is important to point out these cases, as this disease is rare in children and difficult to diagnose because the duration of the episodes is usually limited and, most of them are not evaluated by a physician. Thus, we wish to stress how important it is that the family take photographs during an episode, and we encourage professionals to consider Raynaud's phenomenon when dealing with similar cases.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Ethical Disclosures</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Protection of human and animal subjects</span><p id="par0110" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Confidentiality of data</span><p id="par0115" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work center on the publication of patient data.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Right to privacy and informed consent</span><p id="par0120" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article.</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conflicts of Interest</span><p id="par0125" class="elsevierStylePara elsevierViewall">The authors declare they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres752432" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec754821" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres752433" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec754820" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Case Report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:3 [ "identificador" => "sec0020" "titulo" => "Ethical Disclosures" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0025" "titulo" => "Protection of human and animal subjects" ] 1 => array:2 [ "identificador" => "sec0030" "titulo" => "Confidentiality of data" ] 2 => array:2 [ "identificador" => "sec0035" "titulo" => "Right to privacy and informed consent" ] ] ] 8 => array:2 [ "identificador" => "sec0040" "titulo" => "Conflicts of Interest" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2015-06-09" "fechaAceptado" => "2015-08-18" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec754821" "palabras" => array:6 [ 0 => "Raynaud's phenomenon" 1 => "Pediatrics" 2 => "Paleness" 3 => "Blush" 4 => "Cyanosis" 5 => "Capillaroscopy" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec754820" "palabras" => array:6 [ 0 => "Fénomeno de Raynaud" 1 => "Pediatría" 2 => "Palidez" 3 => "Rubor" 4 => "Cianosis" 5 => "Capilaroscopia" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Raynaud's phenomenon is caused by spasm of the small arteries and arterioles of the fingers. It is triggered by various stimuli including exposure to cold or a stressful event. It may be symmetrical or wrap one end. The appearance of this entity in children is rare.</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We report the case of a 4 year old male consultation health center by episodes of coldness, pallor and pain in both feet.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">El fenómeno de Raynaud está causado por vasoespasmo de las pequeñas arterias y arteriolas de los dedos. Se desencadena por diversos estímulos, entre ellos la exposición al frío o un evento estresante. Puede ser simétrico o envolver una sola extremidad. La aparición de esta entidad en niños es muy infrecuente.</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Presentamos el caso de un varón de 4 años que consulta en su centro de salud por episodios de frialdad, palidez y dolor en ambos pies.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Ortega Vicente E, Garrido Redondo M. Fenómeno de Raynaud en pediatría. Reumatol Clin. 2016;12:342–344.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1128 "Ancho" => 900 "Tamanyo" => 118690 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Skin pallor during an episode.</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" => 699 "Ancho" => 995 "Tamanyo" => 86234 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Phase of erythema in both feet.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Raynaud's phenomenon in children: a retrospective review of 123 patients" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "P.A. 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Castillo-Caro" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:4 [ "tituloSerie" => "Pediatr Rheum" "fecha" => "2011" "volumen" => "9" "paginaInicial" => "16" ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0045" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Comparison of wide field nailfold capillaroscopy and videocapillaroscopy in de assessment of the microcirculation in patients with Raynaud's Phenomenon" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "J. Sekiyama" 1 => "C. Camargo" 2 => "L.E. Andrade" 3 => "C. Kayser" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:4 [ "tituloSerie" => "Arthritis Rheum" "fecha" => "2011" "volumen" => "63" "paginaInicial" => "1457" ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0050" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Prospective nailfold capillaroscopy evaluation of Raynaud's phenomenon in children and adolescents" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "D.G.P. Piotto" 1 => "M.O.E. Hilario" 2 => "N.S. Carvalho" 3 => "C.A. Len" 4 => "L.E. Andrade" 5 => "M.T. 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