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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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" ]
| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 17 | 8 | 25 |
| 2024 Octubre | 107 | 45 | 152 |
| 2024 Septiembre | 91 | 32 | 123 |
| 2024 Agosto | 161 | 60 | 221 |
| 2024 Julio | 136 | 42 | 178 |
| 2024 Junio | 128 | 30 | 158 |
| 2024 Mayo | 106 | 52 | 158 |
| 2024 Abril | 114 | 30 | 144 |
| 2024 Marzo | 99 | 43 | 142 |
| 2024 Febrero | 90 | 28 | 118 |
| 2024 Enero | 93 | 36 | 129 |
| 2023 Diciembre | 96 | 44 | 140 |
| 2023 Noviembre | 99 | 45 | 144 |
| 2023 Octubre | 113 | 23 | 136 |
| 2023 Septiembre | 108 | 43 | 151 |
| 2023 Agosto | 71 | 39 | 110 |
| 2023 Julio | 101 | 59 | 160 |
| 2023 Junio | 82 | 30 | 112 |
| 2023 Mayo | 89 | 26 | 115 |
| 2023 Abril | 99 | 25 | 124 |
| 2023 Marzo | 93 | 37 | 130 |
| 2023 Febrero | 68 | 24 | 92 |
| 2023 Enero | 50 | 50 | 100 |
| 2022 Diciembre | 78 | 48 | 126 |
| 2022 Noviembre | 60 | 37 | 97 |
| 2022 Octubre | 58 | 31 | 89 |
| 2022 Septiembre | 34 | 50 | 84 |
| 2022 Agosto | 38 | 47 | 85 |
| 2022 Julio | 34 | 48 | 82 |
| 2022 Junio | 33 | 36 | 69 |
| 2022 Mayo | 50 | 38 | 88 |
| 2022 Abril | 65 | 33 | 98 |
| 2022 Marzo | 72 | 54 | 126 |
| 2022 Febrero | 54 | 34 | 88 |
| 2022 Enero | 69 | 52 | 121 |
| 2021 Diciembre | 56 | 44 | 100 |
| 2021 Noviembre | 59 | 53 | 112 |
| 2021 Octubre | 100 | 60 | 160 |
| 2021 Septiembre | 50 | 37 | 87 |
| 2021 Agosto | 64 | 39 | 103 |
| 2021 Julio | 49 | 27 | 76 |
| 2021 Junio | 32 | 33 | 65 |
| 2021 Mayo | 35 | 37 | 72 |
| 2021 Abril | 68 | 63 | 131 |
| 2021 Marzo | 64 | 31 | 95 |
| 2021 Febrero | 62 | 28 | 90 |
| 2021 Enero | 46 | 22 | 68 |
| 2020 Diciembre | 30 | 21 | 51 |
| 2020 Noviembre | 51 | 33 | 84 |
| 2020 Octubre | 51 | 11 | 62 |
| 2020 Septiembre | 33 | 12 | 45 |
| 2020 Agosto | 32 | 17 | 49 |
| 2020 Julio | 25 | 17 | 42 |
| 2020 Junio | 31 | 25 | 56 |
| 2020 Mayo | 24 | 25 | 49 |
| 2020 Abril | 33 | 26 | 59 |
| 2020 Marzo | 32 | 15 | 47 |
| 2020 Febrero | 1 | 3 | 4 |
| 2020 Enero | 0 | 12 | 12 |
| 2019 Diciembre | 4 | 8 | 12 |
| 2019 Noviembre | 0 | 4 | 4 |
| 2019 Octubre | 0 | 2 | 2 |
| 2019 Septiembre | 4 | 6 | 10 |
| 2019 Agosto | 0 | 3 | 3 |
| 2019 Julio | 0 | 12 | 12 |
| 2019 Junio | 2 | 6 | 8 |
| 2019 Mayo | 0 | 43 | 43 |
| 2019 Abril | 0 | 15 | 15 |
| 2019 Marzo | 2 | 7 | 9 |
| 2019 Febrero | 0 | 4 | 4 |
| 2019 Enero | 2 | 0 | 2 |
| 2018 Diciembre | 1 | 0 | 1 |
| 2018 Noviembre | 2 | 2 | 4 |
| 2018 Octubre | 2 | 0 | 2 |
| 2018 Septiembre | 2 | 0 | 2 |
| 2018 Junio | 0 | 1 | 1 |
| 2018 Marzo | 2 | 2 | 4 |
| 2018 Febrero | 35 | 3 | 38 |
| 2018 Enero | 31 | 8 | 39 |
| 2017 Diciembre | 40 | 10 | 50 |
| 2017 Noviembre | 25 | 4 | 29 |
| 2017 Octubre | 37 | 5 | 42 |
| 2017 Septiembre | 37 | 5 | 42 |
| 2017 Agosto | 41 | 6 | 47 |
| 2017 Julio | 64 | 9 | 73 |
| 2017 Junio | 57 | 13 | 70 |
| 2017 Mayo | 48 | 21 | 69 |
| 2017 Abril | 48 | 13 | 61 |
| 2017 Marzo | 28 | 30 | 58 |
| 2017 Febrero | 27 | 10 | 37 |
| 2017 Enero | 22 | 8 | 30 |
| 2016 Diciembre | 36 | 8 | 44 |
| 2016 Noviembre | 48 | 12 | 60 |
| 2016 Octubre | 70 | 10 | 80 |
| 2016 Septiembre | 72 | 7 | 79 |
| 2016 Agosto | 49 | 9 | 58 |
| 2016 Julio | 41 | 5 | 46 |
| 2016 Junio | 6 | 8 | 14 |
| 2016 Mayo | 7 | 8 | 15 |
| 2016 Abril | 5 | 6 | 11 |
| 2016 Marzo | 5 | 7 | 12 |
| 2016 Febrero | 7 | 1 | 8 |
| 2016 Enero | 9 | 1 | 10 |
| 2015 Diciembre | 13 | 8 | 21 |
| 2015 Noviembre | 15 | 1 | 16 |
| 2015 Octubre | 13 | 14 | 27 |
| 2015 Septiembre | 2 | 5 | 7 |
| 2015 Agosto | 8 | 3 | 11 |
| 2015 Julio | 64 | 5 | 69 |
| 2015 Junio | 47 | 3 | 50 |
| 2015 Mayo | 54 | 6 | 60 |
| 2015 Abril | 48 | 10 | 58 |
| 2015 Marzo | 32 | 8 | 40 |
| 2015 Febrero | 39 | 2 | 41 |
| 2015 Enero | 30 | 4 | 34 |
| 2014 Diciembre | 43 | 3 | 46 |
| 2014 Noviembre | 18 | 5 | 23 |
| 2014 Octubre | 40 | 7 | 47 |
| 2014 Septiembre | 22 | 3 | 25 |
| 2014 Agosto | 31 | 7 | 38 |
| 2014 Julio | 20 | 10 | 30 |
| 2014 Junio | 25 | 5 | 30 |
| 2014 Mayo | 51 | 8 | 59 |
| 2014 Abril | 26 | 5 | 31 |
| 2014 Marzo | 28 | 5 | 33 |
| 2014 Febrero | 23 | 4 | 27 |
| 2014 Enero | 31 | 3 | 34 |
| 2013 Diciembre | 16 | 4 | 20 |
| 2013 Noviembre | 3 | 2 | 5 |
| 2013 Octubre | 2 | 1 | 3 |
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