Case for Diagnosis
Atrophic Plaque on the Back of a 14-Year-Old Boy
Placa atrófica en la espalda de un varón de 14 años
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Esteban Terradillos, I. Allende Markixana, V. Velasco Benito" "autores" => array:3 [ 0 => array:4 [ "nombre" => "S." "apellidos" => "Esteban Terradillos" "email" => array:2 [ 0 => "esara049@gmail.com" 1 => "sara.estebanterradillos@osakidetza.eus" ] "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" => "I." "apellidos" => "Allende Markixana" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "V." "apellidos" => "Velasco Benito" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario Cruces, Barakaldo, Vizcaya, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitario Cruces, Barakaldo, Vizcaya, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Placa atrófica en la espalda de un varón de 14 años" ] ] "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" => 720 "Ancho" => 528 "Tamanyo" => 164560 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Upper panel: original magnification ×4. Lower panel: original magnification ×200.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 14-year-old boy with no medical history of interest was seen for an asymptomatic lesion of stable size that had appeared 2 years earlier. The patient believed that the lesion may have been associated with trauma.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed a sclerotic-looking atrophic plaque of approximately 3<span class="elsevierStyleHsp" style=""></span>cm in size with a central purple macule located in the right costolumbar area (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Histopathology showed a hypercellular tumor with poorly defined borders located in the reticular dermis and extending into the hypodermis. The cells were spindle-shaped and arranged horizontally to the epidermis, with a focal swirling pattern and honeycomb-like fat infiltration (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Immunohistochemistry revealed CD34 expression (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><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">Doppler ultrasound performed prior to biopsy showed a poorly defined, subcutaneous echogenic area (30<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>18<span class="elsevierStyleHsp" style=""></span>mm) of absent flow.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Based on the histological diagnosis the patient underwent magnetic resonance imaging, which revealed a subcutaneous lesion that did not reach deep planes and no locoregional lymphadenopathy.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">What Is Your Diagnosis?</span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Atrophic dermatofibrosarcoma protuberans (DFSP).</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">The tumor was excised with wide surgical margins, achieving tumor-free margins. The patient showed no signs of recurrence after 23 months of follow-up.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">DFSP is a cutaneous mesenchymal neoplasm of intermediate malignancy, characterized by a marked tendency to local recurrence and a low metastatic capacity.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> It accounts for 0.1% of all malignant skin tumors. It predominantly appears between the second and fifth decades of life, although it is thought that the proportion of pediatric cases is underestimated due to late diagnosis.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1,2</span></a> Fortunately, in children this tumor appears to be less aggressive and to recur less frequently.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> It typically manifests as pinkish or brownish plaques on which exophytic nodules appear (as implied by the word <span class="elsevierStyleItalic">protuberans</span>), and is usually located on the trunk. In children, DFSP most commonly affects the lower extremities and acral areas, supporting an etiological role of trauma.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Other clinical and pathological subtypes have been described, including an atrophic variant (which may also correspond to an early stage of a prolonged presentation).<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> This subtype can be difficult to distinguish clinically from morphea, atrophoderma, atrophic scar (as in the present case), anetoderma, aplasia cutis, and lipoatrophy.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1,4</span></a> Histology shows a dermal tumor that is thinner than the healthy dermis and retracts the skin, and consists of monomorphic spindle cells with mild atypia arranged in a storiform pattern.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1,4</span></a> Immunohistochemistry is key to distinguishing this lesion from other tumors such as atrophic dermatofibroma, atypical fibroxanthoma, and undifferentiated pleomorphic sarcoma. DFSP is strongly positive for CD34 and negative for S100, EMA, desmin, and smooth muscle actin.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Most DFSP patients carry a translocation in chromosomes 17 and 22, giving rise to the chimeric gene <span class="elsevierStyleItalic">COL1A1-PDGFB</span>, detection of which by fluorescence in situ hybridization (FISH) or reverse transcriptase polymerase chain reaction (RT-PCR) is highly specific for DFSP.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1,5</span></a> The main treatment for DFSP is complete eradication of the tumor through excision with wide margins; Mohs surgery is considered the treatment of choice.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Imatinib treatment can be considered in cases of unresectable tumors or in advanced stages of the disease.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> After surgery, follow-up every 6 to 12 months for at least 5 years is recommended.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> The prognosis for atrophic DFSP is the same as that for the classical form, but confusion of this tumor with benign atrophic lesions by both patients and doctors means that diagnosis and treatment, and subsequent cure, are often delayed.<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3,4</span></a> The present case underscores the importance of biopsy in cases of slow-growing atrophic plaques of uncertain diagnosis.</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conflicts of Interest</span><p id="par0045" 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" => "Additional Tests" ] 4 => array:3 [ "identificador" => "sec0025" "titulo" => "What Is Your Diagnosis?" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0030" "titulo" => "Diagnosis" ] 1 => array:2 [ "identificador" => "sec0035" "titulo" => "Clinical Course and Treatment" ] 2 => array:2 [ "identificador" => "sec0040" "titulo" => "Comment" ] ] ] 5 => array:2 [ "identificador" => "sec0045" "titulo" => "Conflicts of Interest" ] 6 => array:2 [ "identificador" => "xack497373" "titulo" => "Acknowledgments" ] 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: Esteban Terradillos S, Allende Markixana I, Velasco Benito V. Placa atrófica en la espalda de un varón de 14 años. Actas Dermosifiliogr. 2020;111:413–414.</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" => 452 "Ancho" => 604 "Tamanyo" => 27826 ] ] "descripcion" => array:1 [ "en" => "<p id="spar1005" class="elsevierStyleSimplePara elsevierViewall">Clinical appearance of the lesion.</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" => 720 "Ancho" => 528 "Tamanyo" => 164560 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Upper panel: original magnification ×4. Lower panel: original magnification ×200.</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" => 454 "Ancho" => 604 "Tamanyo" => 174045 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Immunohistochemistry showing positive staining for CD34 (original magnification ×100).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Dermatofibrosarcoma protuberans en la infancia" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "M. Valdivielso-Ramos" 1 => "J.M. Hernanz" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.adengl.2011.12.002" "Revista" => array:6 [ "tituloSerie" => "Actas Dermosifiliogr" "fecha" => "2012" "volumen" => "103" "paginaInicial" => "863" "paginaFinal" => "873" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23154247" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0035" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Dermatofibrosarcoma protuberans in children and adolescents: Clinical presentation, histology, treatment, and review of the literature" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "Y.J. Tsai" 1 => "P.Y. Lin" 2 => "K.Y. Chew" 3 => "Y.C. Chiang" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.bjps.2014.05.031" "Revista" => array:6 [ "tituloSerie" => "J Plast Reconstr Aesthet Surg" "fecha" => "2014" "volumen" => "67" "paginaInicial" => "1222" "paginaFinal" => "1229" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24973861" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0040" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "An unusual variant of atrophic dermatofibrosarcoma protuberans" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "S.S.C. Aragão" 1 => "E.J.D.S. Leite" 2 => "A.E.C. Cardoso" 3 => "R.L.S. Houly" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1590/abd1806-4841.20187049" "Revista" => array:6 [ "tituloSerie" => "An Bras Dermatol" "fecha" => "2018" "volumen" => "93" "paginaInicial" => "282" "paginaFinal" => "284" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/29723383" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0045" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Dermatofibrosarcoma protuberans presenting as a large atrophic plaque on the chest" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "R. Saigusa" 1 => "T. Miyagawa" 2 => "S. Toyama" 3 => "J. Omatsu" 4 => "T. Miyagaki" 5 => "Y. Masui" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.2340/00015555-2800" "Revista" => array:6 [ "tituloSerie" => "Acta Derm Venereol" "fecha" => "2018" "volumen" => "98" "paginaInicial" => "155" "paginaFinal" => "156" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28929166" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0050" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Atrophic dermatofibrosarcoma protuberans with the fusion gene COL1A1-PDGFB detected by RT-PCR using only a single primer pair" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "W.J. Xu" 1 => "J.S. Wang" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Int J Clin Exp Pathol" "fecha" => "2015" "volumen" => "8" "paginaInicial" => "7457" "paginaFinal" => "7463" ] ] ] ] ] ] ] ] ] ] "agradecimientos" => array:1 [ 0 => array:4 [ "identificador" => "xack497373" "titulo" => "Acknowledgments" "texto" => "<p id="par0050" class="elsevierStylePara elsevierViewall">The authors thank Dr. Juan Ayestaran of the Plastic Surgery Service and Dr. Alexandre Nogueira of the Pathological Anatomy Service of the Hospital Universitario de Cruces for their important contributions to the diagnosis and treatment of the patient.</p>" "vista" => "all" ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000011100000005/v3_202012020957/S1578219020301402/v3_202012020957/en/main.assets" "Apartado" => array:4 [ "identificador" => "6154" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Case for Diagnosis" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15782190/0000011100000005/v3_202012020957/S1578219020301402/v3_202012020957/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219020301402?idApp=UINPBA000044" ]
| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 9 | 12 | 21 |
| 2024 October | 76 | 35 | 111 |
| 2024 September | 72 | 27 | 99 |
| 2024 August | 93 | 54 | 147 |
| 2024 July | 66 | 33 | 99 |
| 2024 June | 74 | 27 | 101 |
| 2024 May | 60 | 27 | 87 |
| 2024 April | 66 | 26 | 92 |
| 2024 March | 59 | 30 | 89 |
| 2024 February | 47 | 31 | 78 |
| 2024 January | 48 | 34 | 82 |
| 2023 December | 44 | 19 | 63 |
| 2023 November | 61 | 28 | 89 |
| 2023 October | 59 | 25 | 84 |
| 2023 September | 67 | 30 | 97 |
| 2023 August | 49 | 17 | 66 |
| 2023 July | 55 | 42 | 97 |
| 2023 June | 55 | 28 | 83 |
| 2023 May | 55 | 23 | 78 |
| 2023 April | 14 | 14 | 28 |
| 2023 March | 23 | 33 | 56 |
| 2023 February | 22 | 22 | 44 |
| 2023 January | 21 | 22 | 43 |
| 2022 December | 38 | 35 | 73 |
| 2022 November | 15 | 27 | 42 |
| 2022 October | 26 | 21 | 47 |
| 2022 September | 21 | 43 | 64 |
| 2022 August | 23 | 33 | 56 |
| 2022 July | 23 | 37 | 60 |
| 2022 June | 26 | 29 | 55 |
| 2022 May | 23 | 29 | 52 |
| 2022 April | 24 | 31 | 55 |
| 2022 March | 35 | 52 | 87 |
| 2022 February | 24 | 27 | 51 |
| 2022 January | 29 | 36 | 65 |
| 2021 December | 22 | 34 | 56 |
| 2021 November | 36 | 44 | 80 |
| 2021 October | 50 | 57 | 107 |
| 2021 September | 29 | 30 | 59 |
| 2021 August | 19 | 27 | 46 |
| 2021 July | 29 | 24 | 53 |
| 2021 June | 27 | 31 | 58 |
| 2021 May | 28 | 40 | 68 |
| 2021 April | 69 | 71 | 140 |
| 2021 March | 66 | 32 | 98 |
| 2021 February | 37 | 23 | 60 |
| 2021 January | 29 | 19 | 48 |
| 2020 December | 28 | 11 | 39 |
| 2020 November | 21 | 15 | 36 |
| 2020 October | 31 | 19 | 50 |
| 2020 September | 26 | 13 | 39 |
| 2020 August | 42 | 19 | 61 |
| 2020 July | 56 | 21 | 77 |
| 2020 June | 24 | 24 | 48 |
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