Case and Research Letters
Paraneoplastic Acral Vascular Syndrome
Síndrome vascular acral paraneoplásico
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Rodríguez Martín, E. Guirao Arrabal, R. Jiménez Puya, A. Vélez García-Nieto" "autores" => array:4 [ 0 => array:4 [ "nombre" => "A.M." "apellidos" => "Rodríguez Martín" "email" => array:1 [ 0 => "rodriguezmartinanamaria@gmail.com" ] "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" => "E." "apellidos" => "Guirao Arrabal" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "R." "apellidos" => "Jiménez Puya" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "A." "apellidos" => "Vélez García-Nieto" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario Reina Sofía, Universidad de Córdoba, Instituto Maimónides de Investigación Biomédica de Córdoba (IMIBIC), Córdoba, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Medicina Interna, Hospital Universitario Reina Sofía de Córdoba, Córdoba, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome vascular acral paraneoplásico" ] ] "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" => 996 "Ancho" => 995 "Tamanyo" => 78788 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Chest x-ray with a 4<span class="elsevierStyleHsp" style=""></span>cm mass on the lower lobe of the right lung.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Paraneoplastic acral vascular syndrome involves an association between malignancy and Raynaud phenomenon, gangrene, or acrocyanosis of the fingers. Raynaud phenomenon is a transient digital ischemia resulting from vasoconstriction on exposure to cold temperatures or stressful situations. In its secondary form, it is usually associated with rheumatic disease and rarely occurs as a paraneoplastic phenomenon.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1–3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a 49-year-old man with no personal history of interest who smoked 80 cigarettes and drank 60<span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>g of alcohol per day. He visited the emergency department with sudden-onset cyanosis on the first 3 fingers of the left hand that was preceded by cutaneous pallor with paresthesia and intense pain (<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>). He had no other symptoms that were indicative of systemic disease, and the remainder of the physical examination was unremarkable. His heartbeat was regular, with no murmurs, and the distal pulses were palpable and symmetrical in all 4 extremities. The coagulation study was normal, except for D-dimer, which was >5000<span class="elsevierStyleHsp" style=""></span>ng/mL. Prothrombin time and activated partial prothrombin time were normal. Arterial Doppler ultrasound showed that the blood supply reached the spaces between the fingers of the affected hand. Arteriography revealed a distal occlusion of the radial artery of the second finger, with proximal occlusion of the palmar digital arteries of the second and third fingers. The chest x-ray revealed a 4-cm mass on the right lower lobe (<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>). Fine needle aspiration findings were consistent with giant cell carcinoma of the lung. The extension study revealed no lymph node involvement. Initial treatment was with low-molecular-weight heparin, which led to a slight improvement in the skin symptoms. Therefore, pentoxifylline, acetylsalicylic acid, and intravenous alprostadil were added sequentially, although the results were mediocre. A clear clinical improvement was observed once the tumor was removed, and the Raynaud phenomenon resolved.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Only 33 cases of paraneoplastic acral vascular syndrome have been reported to date. Adenocarcinomas of the lung, ovary, and stomach account for 60% of all neoplasms, which are almost always in advanced stages with metastasis. The remaining neoplasms comprise sarcoma, hematologic neoplasm, and tumors of unknown origin. Skin lesions precede cancer in 50% of cases, as in the patient we report. Little is known about the pathogenic mechanism underlying this syndrome, although vasoconstrictive substances produced by tumor cells have been implicated. Embolism caused by small tumor thrombi may also play a role. In some hematologic neoplasms (eg, multiple myeloma), ischemia is caused by increased blood viscosity secondary to associated cryoglobulinemia. Histopathology shows fibrinoid necrosis in the veins and arteries.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4–6</span></a> Our study of the present case shows that a thorough evaluation of the patient should be performed before Raynaud phenomenon can be classed as primary. The criteria for a diagnosis of secondary Raynaud phenomenon are shown in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>. Unilateral presentation should alert the physician to involvement of the subclavian artery. Doppler ultrasound and arteriography can confirm the diagnosis, as in the present case. Furthermore, since it is found in 80% of cases, gangrene should raise the suspicion of a paraneoplastic origin. Nailfold capillaroscopy is also very useful: dilated and tortuous capillaries point to early forms of systemic sclerosis.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Treatment of primary Raynaud phenomenon or Raynaud phenomenon associated with rheumatic disease is based on vasodilators, mainly calcium antagonists, the most commonly used of which is nifedipine. New drugs, such as phosphodiesterase inhibitors (sildenafil, tadalafil, and cilostazol), have proven effective in severe Raynaud phenomenon. Anticoagulant therapy with heparin could be indicated in cases of persistent critical limb ischemia or suspected occlusive arterial disease. Local digital sympathectomy can be considered when digital ischemia is severe and does not respond to medical treatment. This approach leads to a rapid increase in blood flow in the fingers. Paraneoplastic Raynaud phenomenon is usually refractory to vasodilators and sympathectomy, and symptoms are improved by treating the underlying malignancy. Complete cure has been reported in 48% of patients, with control of the tumor, as in the present case.<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">8–10</span></a> Appropriate diagnosis of paraneoplastic Raynaud phenomenon enables the tumor to be detected early and suitable treatment to be initiated quickly. The condition should be suspected when it appears after age 30 in a patient with no history of autoimmune or vascular disease.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Rodríguez Martín AM, Guirao Arrabal E, Jiménez Puya R, Vélez García-Nieto A. Síndrome vascular acral paraneoplásico. Actas Dermosifiliogr. 2015;106:601–602.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1200 "Ancho" => 900 "Tamanyo" => 243484 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Erythema on the second finger of the left hand.</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" => 996 "Ancho" => 995 "Tamanyo" => 78788 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Chest x-ray with a 4<span class="elsevierStyleHsp" style=""></span>cm mass on the lower lobe of the right lung.</p>" ] ] 2 => 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"><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Characteristic \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Primary Raynaud Phenomenon \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Secondary Raynaud Phenomenon \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Age at onset \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">30 y \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">> 30 y \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Sex \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">> 5-10/1 women \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Variable \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Vasospastic attacks (eg, cold temperature, emotional distress) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Yes \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">No \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Duration of symptoms \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">> 5 y \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">< 5 y \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Frequency of attacks \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Several per day \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Few per day \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Symmetrical attacks on both hands \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Yes \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">No \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Absence of edema, ulceration, and necrosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Yes \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">No \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Symptoms indicative of connective tissue disease \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Yes \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Abnormal capillaroscopy findings \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Yes \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="left" valign="top">Antibodies \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">No \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Yes \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab873594.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Diagnostic Criteria of Raynaud Phenomenon (Modified According to Le Roy Medgester, 1992).</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" => "Raynaud's disease" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "J.C. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 5 | 3 | 8 |
| 2024 Octubre | 98 | 44 | 142 |
| 2024 Septiembre | 122 | 34 | 156 |
| 2024 Agosto | 163 | 82 | 245 |
| 2024 Julio | 150 | 49 | 199 |
| 2024 Junio | 117 | 55 | 172 |
| 2024 Mayo | 94 | 27 | 121 |
| 2024 Abril | 93 | 38 | 131 |
| 2024 Marzo | 114 | 36 | 150 |
| 2024 Febrero | 72 | 24 | 96 |
| 2024 Enero | 79 | 37 | 116 |
| 2023 Diciembre | 70 | 26 | 96 |
| 2023 Noviembre | 76 | 33 | 109 |
| 2023 Octubre | 69 | 27 | 96 |
| 2023 Septiembre | 75 | 29 | 104 |
| 2023 Agosto | 61 | 22 | 83 |
| 2023 Julio | 122 | 46 | 168 |
| 2023 Junio | 180 | 24 | 204 |
| 2023 Mayo | 77 | 35 | 112 |
| 2023 Abril | 82 | 32 | 114 |
| 2023 Marzo | 57 | 35 | 92 |
| 2023 Febrero | 70 | 35 | 105 |
| 2023 Enero | 61 | 50 | 111 |
| 2022 Diciembre | 61 | 40 | 101 |
| 2022 Noviembre | 57 | 35 | 92 |
| 2022 Octubre | 43 | 30 | 73 |
| 2022 Septiembre | 29 | 48 | 77 |
| 2022 Agosto | 36 | 37 | 73 |
| 2022 Julio | 30 | 34 | 64 |
| 2022 Junio | 30 | 43 | 73 |
| 2022 Mayo | 61 | 44 | 105 |
| 2022 Abril | 73 | 40 | 113 |
| 2022 Marzo | 103 | 54 | 157 |
| 2022 Febrero | 65 | 31 | 96 |
| 2022 Enero | 87 | 39 | 126 |
| 2021 Diciembre | 50 | 37 | 87 |
| 2021 Noviembre | 63 | 44 | 107 |
| 2021 Octubre | 71 | 50 | 121 |
| 2021 Septiembre | 66 | 28 | 94 |
| 2021 Agosto | 51 | 34 | 85 |
| 2021 Julio | 55 | 26 | 81 |
| 2021 Junio | 52 | 24 | 76 |
| 2021 Mayo | 60 | 41 | 101 |
| 2021 Abril | 106 | 47 | 153 |
| 2021 Marzo | 92 | 28 | 120 |
| 2021 Febrero | 95 | 37 | 132 |
| 2021 Enero | 46 | 21 | 67 |
| 2020 Diciembre | 53 | 27 | 80 |
| 2020 Noviembre | 52 | 41 | 93 |
| 2020 Octubre | 36 | 35 | 71 |
| 2020 Septiembre | 33 | 21 | 54 |
| 2020 Agosto | 32 | 33 | 65 |
| 2020 Julio | 37 | 34 | 71 |
| 2020 Junio | 37 | 37 | 74 |
| 2020 Mayo | 40 | 39 | 79 |
| 2020 Abril | 36 | 21 | 57 |
| 2020 Marzo | 27 | 34 | 61 |
| 2020 Febrero | 4 | 7 | 11 |
| 2020 Enero | 2 | 7 | 9 |
| 2019 Diciembre | 7 | 17 | 24 |
| 2019 Noviembre | 1 | 11 | 12 |
| 2019 Octubre | 0 | 10 | 10 |
| 2019 Septiembre | 0 | 8 | 8 |
| 2019 Agosto | 0 | 3 | 3 |
| 2019 Julio | 4 | 14 | 18 |
| 2019 Junio | 11 | 28 | 39 |
| 2019 Mayo | 4 | 34 | 38 |
| 2019 Abril | 16 | 48 | 64 |
| 2019 Marzo | 3 | 8 | 11 |
| 2019 Febrero | 6 | 11 | 17 |
| 2019 Enero | 4 | 13 | 17 |
| 2018 Diciembre | 8 | 6 | 14 |
| 2018 Noviembre | 2 | 7 | 9 |
| 2018 Octubre | 7 | 0 | 7 |
| 2018 Septiembre | 5 | 4 | 9 |
| 2018 Agosto | 0 | 7 | 7 |
| 2018 Julio | 0 | 10 | 10 |
| 2018 Junio | 0 | 5 | 5 |
| 2018 Mayo | 0 | 9 | 9 |
| 2018 Abril | 0 | 10 | 10 |
| 2018 Marzo | 2 | 2 | 4 |
| 2018 Febrero | 38 | 10 | 48 |
| 2018 Enero | 34 | 10 | 44 |
| 2017 Diciembre | 42 | 13 | 55 |
| 2017 Noviembre | 19 | 13 | 32 |
| 2017 Octubre | 40 | 8 | 48 |
| 2017 Septiembre | 22 | 18 | 40 |
| 2017 Agosto | 23 | 16 | 39 |
| 2017 Julio | 30 | 15 | 45 |
| 2017 Junio | 30 | 23 | 53 |
| 2017 Mayo | 32 | 32 | 64 |
| 2017 Abril | 36 | 17 | 53 |
| 2017 Marzo | 17 | 29 | 46 |
| 2017 Febrero | 18 | 12 | 30 |
| 2017 Enero | 18 | 20 | 38 |
| 2016 Diciembre | 30 | 22 | 52 |
| 2016 Noviembre | 41 | 30 | 71 |
| 2016 Octubre | 27 | 31 | 58 |
| 2016 Septiembre | 0 | 27 | 27 |
| 2016 Agosto | 0 | 6 | 6 |
| 2016 Julio | 5 | 4 | 9 |
| 2016 Junio | 8 | 1 | 9 |
| 2016 Mayo | 13 | 4 | 17 |
| 2016 Abril | 8 | 9 | 17 |
| 2016 Marzo | 0 | 2 | 2 |
| 2016 Febrero | 1 | 1 | 2 |
| 2016 Enero | 0 | 1 | 1 |
| 2015 Octubre | 0 | 5 | 5 |
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