Case for Diagnosis
Scaly Erythematous Patches in a Patient With Down Syndrome
Placas eritematodescamativas en paciente con síndrome de Down
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Hernández-Ruiz, A. García-Herrera, J. Ferrando" "autores" => array:3 [ 0 => array:4 [ "nombre" => "E." "apellidos" => "Hernández-Ruiz" "email" => array:1 [ 0 => "mehernandezruiz@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" => "A." "apellidos" => "García-Herrera" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "J." 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The lesions left residual hyperpigmentation (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). No other relevant lesions were found.</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">Analysis of a skin biopsy specimen from the edge of a plaque revealed an acanthotic epidermis with hyperkeratosis, without the presence of intraepidermal neutrophils. Transepidermal elimination of dermal basophilic material was also observed (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>); orcein staining revealed that the material consisted of elastic fibers (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>).</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" id="sect0020">Diagnosis</span><p id="par0025" class="elsevierStylePara elsevierViewall">Elastosis perforans serpiginosa.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical Course and Treatment</span><p id="par0030" class="elsevierStylePara elsevierViewall">The patient received treatment with topical betamethasone and calcipotriol, to which the response was poor. Because the lesions were asymptomatic, we decided not to initiate more aggressive treatment.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Elastosis perforans serpiginosa (EPS) is a rare perforating dermatosis that usually appears in the first decades of life, normally before age 30 years, in the form of keratotic papules 2 to 5<span class="elsevierStyleHsp" style=""></span>mm in diameter on the face, neck, trunk, and upper limbs with a symmetrical distribution. The lesions tend to be asymptomatic or mildly pruritic. Although EPS is classically described as affecting the head, neck, and arms, there have been reports of cases affecting the lower limbs, especially the thighs.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1–3</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Histologic examination of the lesions shows hyperkeratosis with or without parakeratosis, as well as the characteristic transepidermal elimination of material that can be identified by orcein staining as elastic fibers.</p><p id="par0045" class="elsevierStylePara elsevierViewall">There are 3 types of EPS: idiopathic, reactive, and drug-induced. Reactive forms account for around 40% of cases and are associated with other diseases involving connective tissue alterations, such as Down syndrome,<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1,3</span></a> Ehlers-Danlos syndrome, Marfan syndrome, Rothmund-Thomson syndrome, and pseudoxanthoma elasticum.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The fact that the incidence of connective tissue alterations such as joint hypermobility and premature skin aging is higher in patients with Down syndrome could explain why EPS lesions are more common in these patients than in the general population. Drug-induced forms of EPS are observed in approximately 1% of patients treated with D-penicillamine,<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> a drug used in the treatment of Wilson disease and homocystinuria.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Many treatments have been used in EPS, with variable responses. Topical treatments such as corticosteroids,<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> retinoids, and calcineurin inhibitors can be used. Various more aggressive ablative treatments have also been used, including laser therapy, cryotherapy, and tangential excision of some lesions, as well as simple destruction of the lesions by electrocautery.</p><p id="par0060" class="elsevierStylePara elsevierViewall">We have presented a case of EPS that is atypical because of the site—the lower limbs—and because of the size of the lesions. Reports of similar cases in patients with Down syndrome lead us to believe that lesions on the lower limbs may be a common clinical presentation of EPS in this subgroup of patients.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:7 [ 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: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: Hernández-Ruiz E, García-Herrera A, Ferrando J. Placas eritematodescamativas en paciente con síndrome de Down. Actas Dermosifiliogr. 2015;106:753–754.</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" => 550 "Ancho" => 735 "Tamanyo" => 107391 ] ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 550 "Ancho" => 840 "Tamanyo" => 130290 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin-eosin, original magnification ×<span class="elsevierStyleHsp" style=""></span>4.</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" => 1000 "Ancho" => 753 "Tamanyo" => 173270 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Orcein, original magnification ×<span class="elsevierStyleHsp" style=""></span>10.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0035" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Elastosis perforans serpiginosa in an adult with Down's syndrome: Report of a case with symmetrical localized involvement" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "R. 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Conta" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Acta Dermatovenerol Alp Pannonica Adriat" "fecha" => "2014" "volumen" => "23" "paginaInicial" => "39" "paginaFinal" => "41" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24964949" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000010600000009/v1_201511040157/S1578219015002565/v1_201511040157/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/15782190/0000010600000009/v1_201511040157/S1578219015002565/v1_201511040157/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219015002565?idApp=UINPBA000044" ]
| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 9 | 8 | 17 |
| 2024 Octubre | 61 | 35 | 96 |
| 2024 Septiembre | 59 | 27 | 86 |
| 2024 Agosto | 95 | 58 | 153 |
| 2024 Julio | 75 | 36 | 111 |
| 2024 Junio | 90 | 35 | 125 |
| 2024 Mayo | 69 | 36 | 105 |
| 2024 Abril | 74 | 23 | 97 |
| 2024 Marzo | 66 | 23 | 89 |
| 2024 Febrero | 49 | 34 | 83 |
| 2024 Enero | 53 | 33 | 86 |
| 2023 Diciembre | 61 | 14 | 75 |
| 2023 Noviembre | 64 | 25 | 89 |
| 2023 Octubre | 56 | 17 | 73 |
| 2023 Septiembre | 78 | 25 | 103 |
| 2023 Agosto | 60 | 13 | 73 |
| 2023 Julio | 82 | 38 | 120 |
| 2023 Junio | 82 | 26 | 108 |
| 2023 Mayo | 50 | 27 | 77 |
| 2023 Abril | 71 | 19 | 90 |
| 2023 Marzo | 87 | 26 | 113 |
| 2023 Febrero | 58 | 31 | 89 |
| 2023 Enero | 55 | 27 | 82 |
| 2022 Diciembre | 53 | 44 | 97 |
| 2022 Noviembre | 37 | 35 | 72 |
| 2022 Octubre | 27 | 24 | 51 |
| 2022 Septiembre | 54 | 35 | 89 |
| 2022 Agosto | 59 | 37 | 96 |
| 2022 Julio | 66 | 37 | 103 |
| 2022 Junio | 31 | 28 | 59 |
| 2022 Mayo | 40 | 38 | 78 |
| 2022 Abril | 30 | 33 | 63 |
| 2022 Marzo | 35 | 45 | 80 |
| 2022 Febrero | 23 | 30 | 53 |
| 2022 Enero | 33 | 36 | 69 |
| 2021 Diciembre | 26 | 49 | 75 |
| 2021 Noviembre | 43 | 49 | 92 |
| 2021 Octubre | 38 | 51 | 89 |
| 2021 Septiembre | 31 | 33 | 64 |
| 2021 Agosto | 36 | 19 | 55 |
| 2021 Julio | 17 | 26 | 43 |
| 2021 Junio | 30 | 18 | 48 |
| 2021 Mayo | 26 | 21 | 47 |
| 2021 Abril | 33 | 63 | 96 |
| 2021 Marzo | 48 | 22 | 70 |
| 2021 Febrero | 44 | 25 | 69 |
| 2021 Enero | 35 | 12 | 47 |
| 2020 Diciembre | 28 | 17 | 45 |
| 2020 Noviembre | 22 | 21 | 43 |
| 2020 Octubre | 40 | 12 | 52 |
| 2020 Septiembre | 33 | 14 | 47 |
| 2020 Agosto | 21 | 16 | 37 |
| 2020 Julio | 28 | 13 | 41 |
| 2020 Junio | 32 | 24 | 56 |
| 2020 Mayo | 23 | 22 | 45 |
| 2020 Abril | 21 | 16 | 37 |
| 2020 Marzo | 30 | 20 | 50 |
| 2020 Febrero | 2 | 3 | 5 |
| 2020 Enero | 0 | 3 | 3 |
| 2019 Diciembre | 2 | 4 | 6 |
| 2019 Septiembre | 4 | 2 | 6 |
| 2019 Julio | 0 | 7 | 7 |
| 2019 Junio | 2 | 3 | 5 |
| 2019 Mayo | 0 | 16 | 16 |
| 2019 Abril | 0 | 9 | 9 |
| 2019 Marzo | 2 | 3 | 5 |
| 2019 Febrero | 0 | 2 | 2 |
| 2019 Enero | 4 | 0 | 4 |
| 2018 Diciembre | 4 | 2 | 6 |
| 2018 Noviembre | 2 | 2 | 4 |
| 2018 Octubre | 3 | 2 | 5 |
| 2018 Septiembre | 10 | 0 | 10 |
| 2018 Febrero | 27 | 2 | 29 |
| 2018 Enero | 32 | 5 | 37 |
| 2017 Diciembre | 44 | 8 | 52 |
| 2017 Noviembre | 26 | 5 | 31 |
| 2017 Octubre | 35 | 6 | 41 |
| 2017 Septiembre | 31 | 7 | 38 |
| 2017 Agosto | 35 | 5 | 40 |
| 2017 Julio | 29 | 4 | 33 |
| 2017 Junio | 51 | 10 | 61 |
| 2017 Mayo | 47 | 4 | 51 |
| 2017 Abril | 28 | 9 | 37 |
| 2017 Marzo | 21 | 24 | 45 |
| 2017 Febrero | 22 | 9 | 31 |
| 2017 Enero | 25 | 8 | 33 |
| 2016 Diciembre | 51 | 11 | 62 |
| 2016 Noviembre | 53 | 21 | 74 |
| 2016 Octubre | 38 | 19 | 57 |
| 2016 Septiembre | 0 | 3 | 3 |
| 2016 Julio | 1 | 1 | 2 |
| 2016 Junio | 3 | 2 | 5 |
| 2016 Mayo | 2 | 3 | 5 |
| 2016 Abril | 0 | 13 | 13 |
| 2016 Febrero | 0 | 1 | 1 |
| 2016 Enero | 0 | 2 | 2 |
| 2015 Diciembre | 0 | 3 | 3 |
| 2015 Noviembre | 0 | 4 | 4 |
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