Case and Research Letters
Linear Dermatomyofibroma
Dermatomiofibroma lineal
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Torres, Á. Revert, L. Terrádez, E. Jordá" "autores" => array:4 [ 0 => array:4 [ "nombre" => "C." "apellidos" => "Torres" "email" => array:1 [ 0 => "catitorresan@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" => "Á." "apellidos" => "Revert" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "L." "apellidos" => "Terrádez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "E." "apellidos" => "Jordá" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Clínico Universitario de Valencia, Valencia, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Clínico Universitario de Valencia, Valencia, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Departamento de Medicina, Universidad de Valencia, Valencia, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Dermatomiofibroma lineal" ] ] "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" => 1424 "Ancho" => 950 "Tamanyo" => 403461 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Dermal proliferation of spindle-shaped cells in interwoven bundles running parallel to the skin surface. Hematoxylin and eosin, original magnification<span class="elsevierStyleHsp" style=""></span>×40. B, Detail of the cells with wavy, elongated nuclei and a rounded outline. Hematoxylin and eosin, original magnification<span class="elsevierStyleHsp" style=""></span>×400.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Dermatomyofibroma is a rare benign tumor of myofibroblastic origin. It occurs predominantly in young women and arises mainly on the upper part of the trunk. In the majority of cases it presents as a small, asymptomatic plaque or nodule with a slightly erythematous surface.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> We present a case of dermatomyofibroma that developed in an elderly man at an unusual site and with uncommon clinical characteristics for this entity.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The patient was a 74-year-old man with a history of in situ melanoma. He consulted for a symptomatic lesion that had appeared 4 months earlier in the right popliteal fossa. Examination revealed an indurated hyperpigmented plaque of 5<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>13<span class="elsevierStyleHsp" style=""></span>cm, with a linear distribution and poorly defined borders (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The patient did not report trauma and described discomfort on flexing the limb. On a suspicion of morphea, the lesion was biopsied. In the mid and deep dermis there was a proliferation of spindle-shaped cells in interwoven bundles, with a tendency to run parallel to the epidermal surface. This proliferation was composed of cells with wavy, elongated nuclei with a rounded outline, no atypia, and with a poorly defined cytoplasm (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Immunohistochemistry was positive for smooth muscle actin, but the cells were negative for CD34, muscle specific actin, and factor XIIIa. Staining with orcein showed increased numbers and fragmentation of the elastic fibers (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). Based on these findings we made a diagnosis of dermatomyofibroma. Surgery was excluded because of the size of the lesion and it was decided to take a wait-and-see approach.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Dermatomyofibroma was first described in 1991 by Hügel, who used the name plaque fibromatosis, but a year later Kamino proposed the term dermatomyofibroma.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> It is a rare benign skin tumor that predominantly affects women. In the largest series, published by Wentzel et al.,<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> the mean age of the patients was 30 years (range, 3 to 51 years). Some cases have also been reported in men, the majority during childhood; the youngest patient to date has been an 11-month-old infant boy.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3,4</span></a> It is thought that in men the lesion may undergo spontaneous regression during puberty, whereas, in women, it grows slowly under the influence of female hormones.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3,4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">From a clinical point of view, the majority of cases present as a palpable plaque or nodule measuring 1 to 2<span class="elsevierStyleHsp" style=""></span>cm in diameter, with a hyperpigmented or erythematous-yellowish surface.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> However, lesions presenting as large linear plaques of 8<span class="elsevierStyleHsp" style=""></span>cm have been reported, and even annular lesions of up to 13<span class="elsevierStyleHsp" style=""></span>cm in diameter.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4–6</span></a> The lesions typically arise on the upper part of the trunk, shoulders, axillas, and anterior abdomen,<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> and there is a characteristic predilection for the posterior cervical region in prepubertal children.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> In the series by Wentzel, 1 lesion was observed in the popliteal fossa, the same site as in our case.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Histology shows a proliferation of spindle-shaped cells in the reticular dermis. The cells are arranged in fascicles that run parallel to the epidermal surface; the skin appendages and epidermis are not affected. The cells present a uniform appearance with abundant eosinophilic cytoplasm and an elongated nucleus, with no atypia or mitoses. The elastic fibers may be increased in number and may be fragmented. Immunohistochemistry should be performed in all cases. The cells are positive for vimentin and often for smooth muscle actin, but are negative for protein S-100, CD34, factor XIIIa, desmin, and caldesmon.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,7</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The importance of this entity is its differentiation from other tumors formed of spindle-shaped cells, such as dermatofibroma and dermatofibrosarcoma protuberans. Dermatofibroma and, in particular, its plaque variant with an atrophic appearance or multiple clustered dermatofibromas, can be similar to our case, but the cells of dermatofibroma are positive for factor XIIIa. Dermatofibrosarcoma protuberans has a storiform histologic pattern and immunohistochemistry is positive for CD34. Other diagnoses that must be ruled out are plaque neurofibroma, which is positive for S-100, hypertrophic scar, characterized by an absence of elastic fibers, and piloleiomyoma, which is positive for caldesmon and desmin. In the literature we found a case of an unclassifiable myofibroblastic cell proliferation with clinical manifestations similar to those of our patient, but with a more prolonged course and larger size of the lesion.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,7–10</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The treatment of choice is excision with adequate surgical margins. No recurrences or distant metastases have been observed. In lesions that have been partially excised or in which conservative management has been applied, no evidence of progression of the lesion has been reported.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">We have described an elderly male patient with a large linear dermatomyofibroma of recent onset in the popliteal fossa.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">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:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of Interest" ] 1 => 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: Torres C, Revert Á, Terrádez L, Jordá E. Dermatomiofibroma lineal. Actas Dermosifiliogr. 2016;107:787–789.</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" => 1067 "Ancho" => 800 "Tamanyo" => 135567 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Indurated, hyperpigmented linear plaque in the right popliteal fossa.</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" => 1424 "Ancho" => 950 "Tamanyo" => 403461 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Dermal proliferation of spindle-shaped cells in interwoven bundles running parallel to the skin surface. Hematoxylin and eosin, original magnification<span class="elsevierStyleHsp" style=""></span>×40. B, Detail of the cells with wavy, elongated nuclei and a rounded outline. Hematoxylin and eosin, original magnification<span class="elsevierStyleHsp" style=""></span>×400.</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" => 1422 "Ancho" => 950 "Tamanyo" => 445693 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A, Immunohistochemistry showing diffuse staining of the tumor cells with smooth muscle actin (SMA). Stain for SMA, original magnification<span class="elsevierStyleHsp" style=""></span>×40. B, Histochemical stain with orcein showing an increase in fragmented reticular fibers. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 9 | 8 | 17 |
| 2024 October | 65 | 51 | 116 |
| 2024 September | 86 | 36 | 122 |
| 2024 August | 130 | 77 | 207 |
| 2024 July | 109 | 39 | 148 |
| 2024 June | 94 | 40 | 134 |
| 2024 May | 92 | 123 | 215 |
| 2024 April | 85 | 24 | 109 |
| 2024 March | 80 | 26 | 106 |
| 2024 February | 120 | 33 | 153 |
| 2024 January | 75 | 39 | 114 |
| 2023 December | 79 | 11 | 90 |
| 2023 November | 73 | 32 | 105 |
| 2023 October | 65 | 24 | 89 |
| 2023 September | 74 | 23 | 97 |
| 2023 August | 53 | 19 | 72 |
| 2023 July | 55 | 41 | 96 |
| 2023 June | 57 | 27 | 84 |
| 2023 May | 52 | 23 | 75 |
| 2023 April | 50 | 21 | 71 |
| 2023 March | 44 | 17 | 61 |
| 2023 February | 51 | 28 | 79 |
| 2023 January | 41 | 29 | 70 |
| 2022 December | 48 | 39 | 87 |
| 2022 November | 34 | 27 | 61 |
| 2022 October | 25 | 26 | 51 |
| 2022 September | 26 | 35 | 61 |
| 2022 August | 25 | 36 | 61 |
| 2022 July | 30 | 37 | 67 |
| 2022 June | 17 | 17 | 34 |
| 2022 May | 39 | 35 | 74 |
| 2022 April | 50 | 29 | 79 |
| 2022 March | 43 | 37 | 80 |
| 2022 February | 39 | 24 | 63 |
| 2022 January | 77 | 52 | 129 |
| 2021 December | 30 | 38 | 68 |
| 2021 November | 43 | 44 | 87 |
| 2021 October | 43 | 66 | 109 |
| 2021 September | 34 | 47 | 81 |
| 2021 August | 68 | 33 | 101 |
| 2021 July | 35 | 35 | 70 |
| 2021 June | 28 | 40 | 68 |
| 2021 May | 53 | 48 | 101 |
| 2021 April | 132 | 92 | 224 |
| 2021 March | 60 | 28 | 88 |
| 2021 February | 46 | 24 | 70 |
| 2021 January | 34 | 17 | 51 |
| 2020 December | 28 | 12 | 40 |
| 2020 November | 39 | 22 | 61 |
| 2020 October | 21 | 11 | 32 |
| 2020 September | 24 | 22 | 46 |
| 2020 August | 20 | 15 | 35 |
| 2020 July | 19 | 23 | 42 |
| 2020 June | 36 | 28 | 64 |
| 2020 May | 15 | 10 | 25 |
| 2020 April | 24 | 17 | 41 |
| 2020 March | 22 | 12 | 34 |
| 2020 February | 4 | 0 | 4 |
| 2020 January | 4 | 0 | 4 |
| 2019 December | 6 | 0 | 6 |
| 2019 November | 4 | 0 | 4 |
| 2019 September | 2 | 0 | 2 |
| 2019 August | 4 | 0 | 4 |
| 2019 July | 4 | 0 | 4 |
| 2019 June | 5 | 0 | 5 |
| 2019 May | 6 | 1 | 7 |
| 2019 April | 2 | 4 | 6 |
| 2019 March | 5 | 0 | 5 |
| 2019 February | 1 | 0 | 1 |
| 2019 January | 6 | 0 | 6 |
| 2018 December | 1 | 0 | 1 |
| 2018 November | 1 | 0 | 1 |
| 2018 October | 3 | 0 | 3 |
| 2018 September | 1 | 0 | 1 |
| 2018 February | 31 | 1 | 32 |
| 2018 January | 42 | 14 | 56 |
| 2017 December | 43 | 11 | 54 |
| 2017 November | 46 | 5 | 51 |
| 2017 October | 33 | 7 | 40 |
| 2017 September | 26 | 4 | 30 |
| 2017 August | 32 | 8 | 40 |
| 2017 July | 51 | 9 | 60 |
| 2017 June | 37 | 10 | 47 |
| 2017 May | 25 | 4 | 29 |
| 2017 April | 26 | 9 | 35 |
| 2017 March | 15 | 8 | 23 |
| 2017 February | 22 | 6 | 28 |
| 2017 January | 14 | 10 | 24 |
| 2016 December | 27 | 25 | 52 |
| 2016 November | 45 | 42 | 87 |
| 2016 October | 4 | 4 | 8 |
Show all
