Case for Diagnosis
Rapidly Growing Ulcer in a Patient With Neurofibromatosis Type I
Úlcera de rápido crecimiento en paciente con neurofibromatosis tipo 1
Servicio de Dermatología, Hospital General Universitario de Alicante, Alicante, Spain
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Toledo-Alberola, J. Guijarro-Llorca" "autores" => array:2 [ 0 => array:4 [ "nombre" => "F." "apellidos" => "Toledo-Alberola" "email" => array:1 [ 0 => "ftoledo@coma.es" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "J." "apellidos" => "Guijarro-Llorca" ] ] "afiliaciones" => array:1 [ 0 => array:1 [ "entidad" => "Servicio de Dermatología, Hospital General Universitario de Alicante, Alicante, Spain" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Úlcera de rápido crecimiento en paciente con neurofibromatosis tipo 1" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 744 "Ancho" => 998 "Tamanyo" => 227988 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin–eosin, original magnification ×400.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 55-year-old man with a history of neurofibromatosis type 1, adrenal pheochromocytoma, primary hypothyroidism (treated with levothyroxine replacement therapy), and chronic myelomonocytic leukemia (diagnosed in 2005 and being treated with azacytidine) presented with an asymptomatic, rapidly growing right pretibial skin lesion that had appeared 2 months earlier.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">The patient had a nonsuppurative ulcer measuring several centimeters in diameter with a purple border. Granulation tissue and a central yellowish membrane were observed (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">A skin biopsy revealed a monomorphic infiltrate of large cells with abundant eosinophilic cytoplasm and prominent nuclei (<a class="elsevierStyleCrossRefs" href="#fig0010">Figs. 2 and 3</a>). Biopsy cultures were positive for <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> and methicillin-resistant <span class="elsevierStyleItalic">Staphylococcus aureus</span>.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What Is Your Diagnosis?</span></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Diagnosis</span><p id="par0025" class="elsevierStylePara elsevierViewall">Skin infiltration due to chronic myelomonocytic leukemia (leukemia cutis).</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">The ulcer continued to grow rapidly, penetrating muscle and bone. The patient was referred to the hematology department and underwent palliative chemotherapy with cytarabine and azacytidine. The disease continued to progress, however, and the patient died 2 months later.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Leukemia cutis is a nonspecific term that refers to a skin infiltration caused by any type of leukemia (myeloid or lymphoid).<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Myeloid sarcoma, primary extramedullary leukemia, or chloromas may be diagnosed in the presence of granulocytic precursors; monocytic precursors, however, indicate monoblastic sarcoma. Leukemia cutis usually develops in patients with systemic disease, but in aleukemic forms, which account for fewer than 10% of cases, skin lesions precede bone marrow or peripheral blood disease.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Chronic myeloproliferative disorders and lymphoproliferative disorders have been described in patients with acute myeloid leukemia (AML). These disorders occur more frequently in AML (in 10%–15% of patients), and the incidence can be as high as 50% in monocytic and myelomonocytic subtypes.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,3</span></a> Leukemia cutis is less common in chronic forms of leukemia; when present, it indicates blastic transformation and is therefore a sign of disease progression. These lesions occur more frequently in children and can appear in 25%–30% of congenital leukemia cases.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The clinical presentation of leukemic skin infiltration is nonspecific and may include single or multiple lesions in the form of papules, plaques, or violaceous nodules that vary in size and usually appear on the lower limbs, head, and neck. Oral mucosal petechial lesions and infiltration of the gums,<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> leonine facies, eczematous lesions, genital ulcers, and panniculitis simulating erythema nodosum<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> are less common. In children it is one of the causes of blueberry muffin baby syndrome.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The most frequent skin manifestations, developing in approximately 40% of patients with leukemia, are leukemids or nonleukemic skin disorders, which are attributable to cytopenia or reactions to medication. These lesions present as purpura (petechiae or ecchymosis), leukocytoclastic vasculitis, neutrophilic dermatosis, and opportunistic infections.</p><p id="par0060" class="elsevierStylePara elsevierViewall">A differential clinical diagnosis for leukemia cutis must rule out skin infections related to immune system compromise, nonlymphatic tumor metastasis, Jessner lymphocytic infiltration, and adnexal tumors. A definitive diagnosis is based on histologic findings.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">The histopathology of leukemia cutis shows a perivascular and periadnexal infiltrate or a dense and diffuse interstitial infiltrate that does not affect the epidermis. In myeloid leukemia this infiltrate is composed of large cells with abundant eosinophilic cytoplasm, large nuclei, and small nucleoli. Abundance of mitotic figures may be observed. Neural, adnexal, and muscle structures are rarely involved.</p><p id="par0070" class="elsevierStylePara elsevierViewall">Leukemia cutis is a local manifestation of an underlying condition, which must therefore be the target of curative treatment. Treatment options include systematic chemotherapy along with specific treatment of the leukemia cutis (surgical removal, psoralen-UV-A, topical nitrogen mustard, and daclizumab).<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> The prognosis is poor, the disease is aggressive, and the rate of survival is low (1-year survival, <10%).</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflict of Interest</span><p id="par0075" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</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" => "Diagnosis" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Clinical Course and Treatment" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Comment" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Conflict of Interest" ] 7 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please cite this article as: Toledo-Alberola F, Guijarro-Llorca J. Úlcera de rápido crecimiento en paciente con neurofibromatosis tipo 1. Actas Dermosifiliogr. 2012;103:63–4.</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" => 669 "Ancho" => 1000 "Tamanyo" => 112823 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Ulcer several centimeters in diameter with a purple border.</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" => 744 "Ancho" => 998 "Tamanyo" => 262629 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin–eosin, original magnification ×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" => 744 "Ancho" => 998 "Tamanyo" => 227988 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin–eosin, 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" => "Leukemia cutis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "J.H. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 6 | 10 | 16 |
| 2024 October | 71 | 35 | 106 |
| 2024 September | 63 | 27 | 90 |
| 2024 August | 101 | 52 | 153 |
| 2024 July | 82 | 28 | 110 |
| 2024 June | 77 | 22 | 99 |
| 2024 May | 75 | 29 | 104 |
| 2024 April | 67 | 24 | 91 |
| 2024 March | 75 | 21 | 96 |
| 2024 February | 61 | 29 | 90 |
| 2024 January | 55 | 26 | 81 |
| 2023 December | 56 | 16 | 72 |
| 2023 November | 66 | 22 | 88 |
| 2023 October | 80 | 19 | 99 |
| 2023 September | 75 | 30 | 105 |
| 2023 August | 46 | 16 | 62 |
| 2023 July | 71 | 29 | 100 |
| 2023 June | 54 | 24 | 78 |
| 2023 May | 64 | 26 | 90 |
| 2023 April | 57 | 26 | 83 |
| 2023 March | 53 | 19 | 72 |
| 2023 February | 56 | 25 | 81 |
| 2023 January | 58 | 32 | 90 |
| 2022 December | 67 | 46 | 113 |
| 2022 November | 20 | 26 | 46 |
| 2022 October | 24 | 22 | 46 |
| 2022 September | 32 | 30 | 62 |
| 2022 August | 24 | 34 | 58 |
| 2022 July | 29 | 33 | 62 |
| 2022 June | 28 | 24 | 52 |
| 2022 May | 59 | 39 | 98 |
| 2022 April | 93 | 25 | 118 |
| 2022 March | 95 | 40 | 135 |
| 2022 February | 101 | 49 | 150 |
| 2022 January | 81 | 42 | 123 |
| 2021 December | 82 | 42 | 124 |
| 2021 November | 68 | 34 | 102 |
| 2021 October | 60 | 47 | 107 |
| 2021 September | 54 | 38 | 92 |
| 2021 August | 39 | 23 | 62 |
| 2021 July | 48 | 25 | 73 |
| 2021 June | 58 | 29 | 87 |
| 2021 May | 61 | 44 | 105 |
| 2021 April | 91 | 61 | 152 |
| 2021 March | 83 | 35 | 118 |
| 2021 February | 70 | 28 | 98 |
| 2021 January | 39 | 18 | 57 |
| 2020 December | 45 | 19 | 64 |
| 2020 November | 27 | 17 | 44 |
| 2020 October | 27 | 20 | 47 |
| 2020 September | 50 | 24 | 74 |
| 2020 August | 27 | 15 | 42 |
| 2020 July | 25 | 17 | 42 |
| 2020 June | 45 | 20 | 65 |
| 2020 May | 25 | 15 | 40 |
| 2020 April | 31 | 16 | 47 |
| 2020 March | 32 | 15 | 47 |
| 2020 February | 3 | 0 | 3 |
| 2020 January | 4 | 1 | 5 |
| 2019 December | 4 | 2 | 6 |
| 2019 November | 4 | 0 | 4 |
| 2019 October | 0 | 1 | 1 |
| 2019 September | 4 | 1 | 5 |
| 2019 August | 4 | 0 | 4 |
| 2019 July | 4 | 1 | 5 |
| 2019 June | 4 | 2 | 6 |
| 2019 May | 4 | 10 | 14 |
| 2019 April | 2 | 7 | 9 |
| 2019 March | 0 | 7 | 7 |
| 2019 February | 2 | 4 | 6 |
| 2018 December | 4 | 0 | 4 |
| 2018 November | 1 | 0 | 1 |
| 2018 October | 2 | 0 | 2 |
| 2018 September | 2 | 0 | 2 |
| 2018 March | 1 | 2 | 3 |
| 2018 February | 72 | 6 | 78 |
| 2018 January | 56 | 9 | 65 |
| 2017 December | 88 | 10 | 98 |
| 2017 November | 41 | 12 | 53 |
| 2017 October | 52 | 5 | 57 |
| 2017 September | 64 | 13 | 77 |
| 2017 August | 67 | 16 | 83 |
| 2017 July | 66 | 13 | 79 |
| 2017 June | 73 | 21 | 94 |
| 2017 May | 66 | 14 | 80 |
| 2017 April | 65 | 12 | 77 |
| 2017 March | 63 | 21 | 84 |
| 2017 February | 64 | 14 | 78 |
| 2017 January | 48 | 9 | 57 |
| 2016 December | 62 | 16 | 78 |
| 2016 November | 81 | 17 | 98 |
| 2016 October | 113 | 15 | 128 |
| 2016 September | 155 | 25 | 180 |
| 2016 August | 73 | 7 | 80 |
| 2016 July | 56 | 14 | 70 |
| 2016 June | 10 | 18 | 28 |
| 2016 May | 8 | 6 | 14 |
| 2016 April | 9 | 9 | 18 |
| 2016 March | 9 | 8 | 17 |
| 2016 February | 5 | 9 | 14 |
| 2016 January | 5 | 7 | 12 |
| 2015 December | 10 | 12 | 22 |
| 2015 November | 31 | 1 | 32 |
| 2015 October | 44 | 3 | 47 |
| 2015 September | 20 | 1 | 21 |
| 2015 August | 13 | 6 | 19 |
| 2015 July | 184 | 8 | 192 |
| 2015 June | 93 | 11 | 104 |
| 2015 May | 93 | 23 | 116 |
| 2015 April | 87 | 12 | 99 |
| 2015 March | 75 | 9 | 84 |
| 2015 February | 55 | 13 | 68 |
| 2015 January | 67 | 9 | 76 |
| 2014 December | 54 | 9 | 63 |
| 2014 November | 41 | 7 | 48 |
| 2014 October | 52 | 14 | 66 |
| 2014 September | 62 | 14 | 76 |
| 2014 August | 62 | 15 | 77 |
| 2014 July | 60 | 13 | 73 |
| 2014 June | 92 | 8 | 100 |
| 2014 May | 74 | 16 | 90 |
| 2014 April | 64 | 13 | 77 |
| 2014 March | 80 | 16 | 96 |
| 2014 February | 60 | 12 | 72 |
| 2014 January | 65 | 15 | 80 |
| 2013 December | 32 | 5 | 37 |
| 2013 November | 22 | 7 | 29 |
| 2013 October | 17 | 2 | 19 |
| 2013 September | 14 | 7 | 21 |
| 2013 August | 14 | 5 | 19 |
| 2013 July | 16 | 8 | 24 |
| 2013 June | 19 | 10 | 29 |
| 2013 May | 15 | 3 | 18 |
| 2013 April | 14 | 8 | 22 |
| 2013 March | 17 | 4 | 21 |
| 2013 February | 26 | 6 | 32 |
| 2013 January | 46 | 9 | 55 |
| 2012 December | 25 | 4 | 29 |
| 2012 November | 1 | 1 | 2 |
| 2012 October | 1 | 2 | 3 |
| 2012 September | 0 | 2 | 2 |
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