Case for Diagnosis
Smoker With Raynaud Phenomenon and Painful Nodules on the Legs
Fumador con fenómeno de Raynaud y nódulos dolorosos en las piernas
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This condition had developed several months earlier after the appearance of painful migratory lesions on the lower limbs.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">On examination, erythematous nodules were observed in a linear distribution on both ankles (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>), and there were violaceous macular lesions with a necrotic appearance on the pads of the fingers (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>.)</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Biopsy of one of the nodules revealed a medium-sized vessel in the superficial subcutaneous tissue, with an occlusive intraluminal thrombus. There was a mixed inflammatory infiltrate in the vessel wall and the adjacent fat (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>A), and a granulomatous reaction with multinucleated giant cells at the periphery of the thrombus (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>B). Orcein staining revealed elastic fibers throughout the full thickness of the vessel wall, confirming it to be a vein (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>C).</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Capillaroscopy was normal. Laboratory tests (blood and urine, basic biochemistry, viral serology, tumor markers, tests for hypercoagulability, and screening for autoimmune disease and cyroglobulins) were negative or normal and echocardiography was unremarkable. Arteriography was requested.</p><p id="par0025" class="elsevierStylePara elsevierViewall">¿What Is Your Diagnosis?</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Thromboangiitis obliterans (Buerger disease).</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Arteriography of the upper limbs revealed occlusion of both ulnar arteries; the palmar arch arising from the radial arteries was incomplete and there was a near total absence of distal vasculature. The arterial study revealed no involvement above the elbow. The patient stopped smoking and the lesions gradually improved.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Thromboangiitis obliterans is a segmental, nonatherosclerotic, occlusive inflammatory vascular disease of the small and medium-sized arteries and veins of the extremities. This disease primarily affects young male smokers and is most prevalent in Asian and Eastern Europe populations.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Though its pathogenesis is unclear, tobacco use is central to the initiation and progression of the disease. Thromboangiitis obliterans is considered a smoking-related autoimmune process that affects genetically predisposed patients.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Clinical manifestations are due to distal ischemia, and the main symptom is pain at rest. The disease presents as painful digital ulcers accompanied by cyanosis and coldness, which can progress to gangrene and require amputation of the affected limb. Some cases, including the one described here, are suggestive of a connective tissue disease, as they initiate with arthritis and Raynaud phenomenon.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Raynaud phenomenon and/or migratory thrombophlebitis of superficial veins occur in 40% of patients. Although rare, the visceral arteries (mesenteric, coronary, retinal, cerebral, pulmonary, or renal) may be affected.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Biopsy, which will reveal an inflammatory occlusive thrombus, is rarely indicated. The biopsy is most likely to be diagnostic in the acute phase of the disease and in a vessel from an area of superficial thrombophlebitis,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> as occurred in the present case.</p><p id="par0060" class="elsevierStylePara elsevierViewall">In cases with no pathognomonic signs or symptoms, the diagnosis is one of exclusion.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Although no clear consensus has been reached, a number of diagnostic criteria have been proposed: young male smoker (under 45 years of age) with signs and symptoms of distal ischemia of the extremities (intermittent claudication, pain at rest, ischemic ulcers, or gangrene), absence of connective tissue disease, hypercoagulability or diabetes mellitus, the exclusion a proximal source of emboli by echocardiography, and a compatible arteriography.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Arteriography is characterized by segmental lesions with abrupt arterial occlusion, gradual tapering (mouse tail) of the lumen, and collateral circulation with ‘corkscrew’ morphology.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Cessation of smoking is the only means of slowing the progression of the disease and avoiding amputation.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Even the use of nicotine patches or gum may be sufficient to maintain disease activity. The goal of treatment in refractory cases is to relieve intractable pain and avoid amputation by debridement and the administration of analgesics, antiplatelet drugs, and antibiotics. Alternative approaches include prostaglandin infusion, sympathectomy, revascularization (a very limited technique due to poor distal beds), and the administration of immunosuppressants and vascular endothelial growth factor analogs. The response is variable.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:8 [ 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 and Treatment" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Comment" ] 7 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Floristán M, et al. Fumador con fenómeno de Raynaud y nódulos dolorosos en las piernas. Actas Dermosifiliogr. 2013;104:717–8.</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" => 500 "Ancho" => 666 "Tamanyo" => 72900 ] ] ] 1 => array:6 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 500 "Ancho" => 666 "Tamanyo" => 54584 ] ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 600 "Ancho" => 800 "Tamanyo" => 275389 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Hematoxylin-eosin, original magnification ×100. B, Hematoxylin-eosin, original magnification ×400. C, Orcein stain, original magnification ×400.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Thromboangiitis obliterans (Buerger's disease)" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "J.W. 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Fiessinger" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Rheumatology (Oxford)" "fecha" => "2007" "volumen" => "46" "paginaInicial" => "192" "paginaFinal" => "199" ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Buerger's disease (thromboangiitis obliterans)" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "R. Goiriz-Valdés" 1 => "J. Fernández-Herrera" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Actas Dermosifiliogr" "fecha" => "2005" "volumen" => "96" "paginaInicial" => "553" "paginaFinal" => "562" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16476298" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Thromboangiitis obliterans (Buerger's disease)" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "J.W. Olin" 1 => "A. Shih" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Curr Opin Rheumatol" "fecha" => "2006" "volumen" => "18" "paginaInicial" => "18" "paginaFinal" => "24" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16344615" "web" => "Medline" ] ] ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib0030" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Buerger's disease" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "P.T. Dargon" 1 => "G.J. Landry" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.avsg.2011.11.005" "Revista" => array:6 [ "tituloSerie" => "Ann Vasc Surg" "fecha" => "2012" "volumen" => "26" "paginaInicial" => "871" "paginaFinal" => "880" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22284771" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000010400000008/v1_201309240048/S1578219013001819/v1_201309240048/en/main.assets" "Apartado" => array:4 [ "identificador" => "6171" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Cases for Diagnosis" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15782190/0000010400000008/v1_201309240048/S1578219013001819/v1_201309240048/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219013001819?idApp=UINPBA000044" ]
| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 17 | 8 | 25 |
| 2024 October | 107 | 45 | 152 |
| 2024 September | 91 | 32 | 123 |
| 2024 August | 161 | 60 | 221 |
| 2024 July | 136 | 42 | 178 |
| 2024 June | 128 | 30 | 158 |
| 2024 May | 106 | 52 | 158 |
| 2024 April | 114 | 30 | 144 |
| 2024 March | 99 | 43 | 142 |
| 2024 February | 90 | 28 | 118 |
| 2024 January | 93 | 36 | 129 |
| 2023 December | 96 | 44 | 140 |
| 2023 November | 99 | 45 | 144 |
| 2023 October | 113 | 23 | 136 |
| 2023 September | 108 | 43 | 151 |
| 2023 August | 71 | 39 | 110 |
| 2023 July | 101 | 59 | 160 |
| 2023 June | 82 | 30 | 112 |
| 2023 May | 89 | 26 | 115 |
| 2023 April | 99 | 25 | 124 |
| 2023 March | 93 | 37 | 130 |
| 2023 February | 68 | 24 | 92 |
| 2023 January | 50 | 50 | 100 |
| 2022 December | 78 | 48 | 126 |
| 2022 November | 60 | 37 | 97 |
| 2022 October | 58 | 31 | 89 |
| 2022 September | 34 | 50 | 84 |
| 2022 August | 38 | 47 | 85 |
| 2022 July | 34 | 48 | 82 |
| 2022 June | 33 | 36 | 69 |
| 2022 May | 50 | 38 | 88 |
| 2022 April | 65 | 33 | 98 |
| 2022 March | 72 | 54 | 126 |
| 2022 February | 54 | 34 | 88 |
| 2022 January | 69 | 52 | 121 |
| 2021 December | 56 | 44 | 100 |
| 2021 November | 59 | 53 | 112 |
| 2021 October | 100 | 60 | 160 |
| 2021 September | 50 | 37 | 87 |
| 2021 August | 64 | 39 | 103 |
| 2021 July | 49 | 27 | 76 |
| 2021 June | 32 | 33 | 65 |
| 2021 May | 35 | 37 | 72 |
| 2021 April | 68 | 63 | 131 |
| 2021 March | 64 | 31 | 95 |
| 2021 February | 62 | 28 | 90 |
| 2021 January | 46 | 22 | 68 |
| 2020 December | 30 | 21 | 51 |
| 2020 November | 51 | 33 | 84 |
| 2020 October | 51 | 11 | 62 |
| 2020 September | 33 | 12 | 45 |
| 2020 August | 32 | 17 | 49 |
| 2020 July | 25 | 17 | 42 |
| 2020 June | 31 | 25 | 56 |
| 2020 May | 24 | 25 | 49 |
| 2020 April | 33 | 26 | 59 |
| 2020 March | 32 | 15 | 47 |
| 2020 February | 1 | 3 | 4 |
| 2020 January | 0 | 12 | 12 |
| 2019 December | 4 | 8 | 12 |
| 2019 November | 0 | 4 | 4 |
| 2019 October | 0 | 2 | 2 |
| 2019 September | 4 | 6 | 10 |
| 2019 August | 0 | 3 | 3 |
| 2019 July | 0 | 12 | 12 |
| 2019 June | 2 | 6 | 8 |
| 2019 May | 0 | 43 | 43 |
| 2019 April | 0 | 15 | 15 |
| 2019 March | 2 | 7 | 9 |
| 2019 February | 0 | 4 | 4 |
| 2019 January | 2 | 0 | 2 |
| 2018 December | 1 | 0 | 1 |
| 2018 November | 2 | 2 | 4 |
| 2018 October | 2 | 0 | 2 |
| 2018 September | 2 | 0 | 2 |
| 2018 June | 0 | 1 | 1 |
| 2018 March | 2 | 2 | 4 |
| 2018 February | 35 | 3 | 38 |
| 2018 January | 31 | 8 | 39 |
| 2017 December | 40 | 10 | 50 |
| 2017 November | 25 | 4 | 29 |
| 2017 October | 37 | 5 | 42 |
| 2017 September | 37 | 5 | 42 |
| 2017 August | 41 | 6 | 47 |
| 2017 July | 64 | 9 | 73 |
| 2017 June | 57 | 13 | 70 |
| 2017 May | 48 | 21 | 69 |
| 2017 April | 48 | 13 | 61 |
| 2017 March | 28 | 30 | 58 |
| 2017 February | 27 | 10 | 37 |
| 2017 January | 22 | 8 | 30 |
| 2016 December | 36 | 8 | 44 |
| 2016 November | 48 | 12 | 60 |
| 2016 October | 70 | 10 | 80 |
| 2016 September | 72 | 7 | 79 |
| 2016 August | 49 | 9 | 58 |
| 2016 July | 41 | 5 | 46 |
| 2016 June | 6 | 8 | 14 |
| 2016 May | 7 | 8 | 15 |
| 2016 April | 5 | 6 | 11 |
| 2016 March | 5 | 7 | 12 |
| 2016 February | 7 | 1 | 8 |
| 2016 January | 9 | 1 | 10 |
| 2015 December | 13 | 8 | 21 |
| 2015 November | 15 | 1 | 16 |
| 2015 October | 13 | 14 | 27 |
| 2015 September | 2 | 5 | 7 |
| 2015 August | 8 | 3 | 11 |
| 2015 July | 64 | 5 | 69 |
| 2015 June | 47 | 3 | 50 |
| 2015 May | 54 | 6 | 60 |
| 2015 April | 48 | 10 | 58 |
| 2015 March | 32 | 8 | 40 |
| 2015 February | 39 | 2 | 41 |
| 2015 January | 30 | 4 | 34 |
| 2014 December | 43 | 3 | 46 |
| 2014 November | 18 | 5 | 23 |
| 2014 October | 40 | 7 | 47 |
| 2014 September | 22 | 3 | 25 |
| 2014 August | 31 | 7 | 38 |
| 2014 July | 20 | 10 | 30 |
| 2014 June | 25 | 5 | 30 |
| 2014 May | 51 | 8 | 59 |
| 2014 April | 26 | 5 | 31 |
| 2014 March | 28 | 5 | 33 |
| 2014 February | 23 | 4 | 27 |
| 2014 January | 31 | 3 | 34 |
| 2013 December | 16 | 4 | 20 |
| 2013 November | 3 | 2 | 5 |
| 2013 October | 2 | 1 | 3 |
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