Case and Research Letter
Ultrasound Monitoring of Childhood Linear Morphea
Monitorización ecográfica de la morfea lineal de la infancia
I. Pérez-López
, C. Garrido-Colmenero, R. Ruiz-Villaverde, J. Tercedor-Sánchez
Autor para correspondencia
Unidad de Gestión Clínica de Dermatología Médico Quirúrgica y Venereología, Hospital Universitario Virgen de las Nieves, Granada, Spain
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Pérez-López, C. Garrido-Colmenero, R. Ruiz-Villaverde, J. Tercedor-Sánchez" "autores" => array:4 [ 0 => array:4 [ "nombre" => "I." "apellidos" => "Pérez-López" "email" => array:1 [ 0 => "ipl_elmadrono@hotmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "C." "apellidos" => "Garrido-Colmenero" ] 2 => array:2 [ "nombre" => "R." "apellidos" => "Ruiz-Villaverde" ] 3 => array:2 [ "nombre" => "J." "apellidos" => "Tercedor-Sánchez" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Unidad de Gestión Clínica de Dermatología Médico Quirúrgica y Venereología, Hospital Universitario Virgen de las Nieves, Granada, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Monitorización ecográfica de la morfea lineal de la infancia" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1244 "Ancho" => 1659 "Tamanyo" => 207397 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Depressed linear lesion on the left upper lip in our patient.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Morphea is a form of localized scleroderma that differs from systemic forms of the disease by the presence of well-characterized morphological variants and the absence of any clinically detectable extracutaneous involvement. Many clinical classifications are employed, but there are few management guidelines for use in daily clinical practice.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> The most recently published guideline includes the following clinical variants: circumscribed morphea, generalized morphea, and the linear variant, which is more typical in childhood.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> The linear variant is subdivided into 3 subtypes: the purely linear form, coup de sabre morphea, and progressive facial hemiatrophy (or Parry-Romberg syndrome).</p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a 7-year-old boy with no past medical or family history of interest. He was seen for a 7-mm long, depressed, hypopigmented, slightly indurated band located on the left upper lip (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The lesion had appeared more than 18 months earlier and had not previously been treated.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">The possibility of performing a diagnostic biopsy was discussed with the family, but, given the cosmetic repercussion, it was decided to use skin ultrasound to confirm the clinical diagnostic suspicion. The ultrasound device employed was the Mylab™25 (Esaote) with a compact linear 18<span class="elsevierStyleHsp" style=""></span>MHz probe. The study was used to support the clinical diagnosis and in particular to ensure correct monitoring of the disease. To perform an accurate study of dermal and epidermal thickness requires a thick layer of gel to obtain a high-quality image; pressure must not be applied to the skin, as this could distort the image. The ultrasound image revealed thinning of the epidermal layer and of the dermosubdermal space, with increased echogenicity of the dermis compared with the adjacent skin (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Doppler mode did not reveal increased vascularization in the area of the lesion. The neurological examination was rigorously normal, and additional laboratory tests were not requested. A diagnosis of inactive linear morphea was made on the basis of the clinical appearance and a compatible ultrasound image, and it was decided to take a wait-and-see approach.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Localized linear scleroderma is characterized by the presence of sclerotic bands with a linear distribution and hypo- or hyperpigmentation; the bands usually arise on the upper or lower limbs. These linear lesions may follow the Blaschko lines, signifying that genetic mosaicism may contribute to the pathophysiology of the disease.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> Large, deep lesions may be associated with muscle and bone atrophy and functional limitation due to joint involvement.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Ultrasound is a useful method for the diagnosis and, in particular, for the follow-up of diseases that affect the dermis and subcutaneous cellular tissue.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,5</span></a> Although the ultrasound findings are sometimes nonspecific, as occurred in our case, they can be very useful when combined with the patient's clinical manifestations to clarify the differential diagnosis. Most research into linear scleroderma reported in the literature describes the use of 20<span class="elsevierStyleHsp" style=""></span>MHz probes, as they reach a maximum depth of 10<span class="elsevierStyleHsp" style=""></span>mm, which should be sufficient to determine the thickness and echogenicity of the lesion.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Evaluation of the clinical activity of localized scleroderma can be complicated, as it is based on observation of the clinical manifestations: presence or absence of erythema, lesion spread, or appearance of new lesions.</p><p id="par0035" class="elsevierStylePara elsevierViewall">However, the progression of morphea can initially affect deeper layers and be clinically undetectable. This can delay the early initiation of treatment.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The difference in thickness between affected skin and normal skin does not correlate with disease activity. The different characteristics that should be taken into consideration are overall echogenicity of the lesion, hypoechogenicity of the hypodermis, and increased vascularization of the deep dermis. These parameters, which do appear to correlate with increased clinical activity,<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> were evaluated in our patient and enabled us to take a wait-and-see approach. When morphea is in a clinically stable phase, skin ultrasound reveals only a minimal difference in overall echogenicity between affected skin and normal skin, as was found in our patient.<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">9,10</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">In conclusion, skin ultrasound is a non-invasive technique that can reduce the need for skin biopsy, particularly when the clinical findings are highly suggestive, as in our patient, or when histology has already been performed, avoiding cosmetic repercussions in visible areas. The dermatologist must learn to perform ultrasound studies, as they enable plaques of morphea to be monitored and contribute not only to the ability to take a wait-and-see approach but also to monitoring the efficacy of treatments.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Pérez-López I, Garrido-Colmenero C, Ruiz-Villaverde R, Tercedor-Sánchez J. Monitorización ecográfica de la morfea lineal de la infancia. Actas Dermosifiliogr. 2015;106:340–342.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1244 "Ancho" => 1659 "Tamanyo" => 207397 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Depressed linear lesion on the left upper lip in our patient.</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" => 1148 "Ancho" => 1661 "Tamanyo" => 142677 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Reduced thickness of the epidermal layer and of the dermosubdermal space with increased echogenicity of the dermis compared with the adjacent skin of normal appearance. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 7 | 8 | 15 |
| 2024 Octubre | 82 | 42 | 124 |
| 2024 Septiembre | 91 | 23 | 114 |
| 2024 Agosto | 120 | 59 | 179 |
| 2024 Julio | 123 | 48 | 171 |
| 2024 Junio | 101 | 44 | 145 |
| 2024 Mayo | 103 | 35 | 138 |
| 2024 Abril | 113 | 27 | 140 |
| 2024 Marzo | 78 | 50 | 128 |
| 2024 Febrero | 77 | 30 | 107 |
| 2024 Enero | 68 | 34 | 102 |
| 2023 Diciembre | 68 | 11 | 79 |
| 2023 Noviembre | 79 | 35 | 114 |
| 2023 Octubre | 83 | 31 | 114 |
| 2023 Septiembre | 63 | 25 | 88 |
| 2023 Agosto | 54 | 20 | 74 |
| 2023 Julio | 117 | 48 | 165 |
| 2023 Junio | 67 | 24 | 91 |
| 2023 Mayo | 88 | 23 | 111 |
| 2023 Abril | 120 | 24 | 144 |
| 2023 Marzo | 107 | 28 | 135 |
| 2023 Febrero | 99 | 31 | 130 |
| 2023 Enero | 86 | 30 | 116 |
| 2022 Diciembre | 79 | 38 | 117 |
| 2022 Noviembre | 70 | 26 | 96 |
| 2022 Octubre | 60 | 29 | 89 |
| 2022 Septiembre | 48 | 37 | 85 |
| 2022 Agosto | 36 | 25 | 61 |
| 2022 Julio | 32 | 28 | 60 |
| 2022 Junio | 40 | 19 | 59 |
| 2022 Mayo | 106 | 34 | 140 |
| 2022 Abril | 104 | 31 | 135 |
| 2022 Marzo | 83 | 40 | 123 |
| 2022 Febrero | 74 | 24 | 98 |
| 2022 Enero | 120 | 30 | 150 |
| 2021 Diciembre | 84 | 41 | 125 |
| 2021 Noviembre | 78 | 40 | 118 |
| 2021 Octubre | 75 | 50 | 125 |
| 2021 Septiembre | 64 | 30 | 94 |
| 2021 Agosto | 127 | 27 | 154 |
| 2021 Julio | 80 | 29 | 109 |
| 2021 Junio | 62 | 22 | 84 |
| 2021 Mayo | 74 | 19 | 93 |
| 2021 Abril | 100 | 36 | 136 |
| 2021 Marzo | 110 | 15 | 125 |
| 2021 Febrero | 76 | 31 | 107 |
| 2021 Enero | 38 | 26 | 64 |
| 2020 Diciembre | 55 | 15 | 70 |
| 2020 Noviembre | 30 | 16 | 46 |
| 2020 Octubre | 36 | 11 | 47 |
| 2020 Septiembre | 40 | 19 | 59 |
| 2020 Agosto | 39 | 17 | 56 |
| 2020 Julio | 31 | 14 | 45 |
| 2020 Junio | 55 | 26 | 81 |
| 2020 Mayo | 25 | 23 | 48 |
| 2020 Abril | 43 | 23 | 66 |
| 2020 Marzo | 30 | 14 | 44 |
| 2020 Febrero | 9 | 1 | 10 |
| 2020 Enero | 4 | 2 | 6 |
| 2019 Diciembre | 8 | 2 | 10 |
| 2019 Noviembre | 4 | 2 | 6 |
| 2019 Octubre | 0 | 3 | 3 |
| 2019 Septiembre | 10 | 3 | 13 |
| 2019 Agosto | 4 | 3 | 7 |
| 2019 Julio | 2 | 4 | 6 |
| 2019 Junio | 4 | 6 | 10 |
| 2019 Mayo | 4 | 32 | 36 |
| 2019 Abril | 0 | 11 | 11 |
| 2019 Marzo | 0 | 5 | 5 |
| 2019 Febrero | 2 | 0 | 2 |
| 2019 Enero | 1 | 1 | 2 |
| 2018 Noviembre | 2 | 0 | 2 |
| 2018 Octubre | 2 | 0 | 2 |
| 2018 Septiembre | 5 | 1 | 6 |
| 2018 Agosto | 0 | 12 | 12 |
| 2018 Julio | 0 | 5 | 5 |
| 2018 Junio | 0 | 6 | 6 |
| 2018 Mayo | 0 | 6 | 6 |
| 2018 Abril | 0 | 6 | 6 |
| 2018 Marzo | 2 | 1 | 3 |
| 2018 Febrero | 29 | 5 | 34 |
| 2018 Enero | 51 | 6 | 57 |
| 2017 Diciembre | 52 | 12 | 64 |
| 2017 Noviembre | 47 | 6 | 53 |
| 2017 Octubre | 35 | 3 | 38 |
| 2017 Septiembre | 37 | 10 | 47 |
| 2017 Agosto | 59 | 12 | 71 |
| 2017 Julio | 52 | 13 | 65 |
| 2017 Junio | 54 | 15 | 69 |
| 2017 Mayo | 56 | 14 | 70 |
| 2017 Abril | 43 | 8 | 51 |
| 2017 Marzo | 29 | 8 | 37 |
| 2017 Febrero | 25 | 17 | 42 |
| 2017 Enero | 24 | 20 | 44 |
| 2016 Diciembre | 43 | 18 | 61 |
| 2016 Noviembre | 43 | 23 | 66 |
| 2016 Octubre | 36 | 15 | 51 |
| 2016 Septiembre | 0 | 4 | 4 |
| 2016 Agosto | 0 | 1 | 1 |
| 2016 Julio | 8 | 1 | 9 |
| 2016 Junio | 11 | 7 | 18 |
| 2016 Mayo | 3 | 13 | 16 |
| 2016 Abril | 10 | 1 | 11 |
| 2016 Marzo | 9 | 3 | 12 |
| 2016 Febrero | 14 | 2 | 16 |
| 2016 Enero | 15 | 13 | 28 |
| 2015 Diciembre | 8 | 3 | 11 |
| 2015 Noviembre | 5 | 5 | 10 |
| 2015 Octubre | 0 | 1 | 1 |
| 2015 Septiembre | 0 | 2 | 2 |
| 2015 Agosto | 0 | 3 | 3 |
| 2015 Julio | 7 | 2 | 9 |
| 2015 Junio | 7 | 9 | 16 |
| 2015 Mayo | 6 | 6 | 12 |
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