Case for Diagnosis
Scaly Erythematous Patches in a Patient With Down Syndrome
Placas eritematodescamativas en paciente con síndrome de Down
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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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Cho" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.5021/ad.2010.22.4.468" "Revista" => array:6 [ "tituloSerie" => "Ann Dermatol" "fecha" => "2010" "volumen" => "22" "paginaInicial" => "468" "paginaFinal" => "471" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21165224" "web" => "Medline" ] ] ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib0060" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Elastosis perforans serpiginosa: A case successfully treated with intralesional steroids and topical allium cepa-allantoin-pentaglycan gel" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. Campanati" 1 => "E. Martina" 2 => "K. Giuliodori" 3 => "G. Ganzetti" 4 => "B. Marconi" 5 => "I. 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" ]
| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 6 | 8 | 14 |
| 2024 October | 61 | 35 | 96 |
| 2024 September | 59 | 27 | 86 |
| 2024 August | 95 | 58 | 153 |
| 2024 July | 75 | 36 | 111 |
| 2024 June | 90 | 35 | 125 |
| 2024 May | 69 | 36 | 105 |
| 2024 April | 74 | 23 | 97 |
| 2024 March | 66 | 23 | 89 |
| 2024 February | 49 | 34 | 83 |
| 2024 January | 53 | 33 | 86 |
| 2023 December | 61 | 14 | 75 |
| 2023 November | 64 | 25 | 89 |
| 2023 October | 56 | 17 | 73 |
| 2023 September | 78 | 25 | 103 |
| 2023 August | 60 | 13 | 73 |
| 2023 July | 82 | 38 | 120 |
| 2023 June | 82 | 26 | 108 |
| 2023 May | 50 | 27 | 77 |
| 2023 April | 71 | 19 | 90 |
| 2023 March | 87 | 26 | 113 |
| 2023 February | 58 | 31 | 89 |
| 2023 January | 55 | 27 | 82 |
| 2022 December | 53 | 44 | 97 |
| 2022 November | 37 | 35 | 72 |
| 2022 October | 27 | 24 | 51 |
| 2022 September | 54 | 35 | 89 |
| 2022 August | 59 | 37 | 96 |
| 2022 July | 66 | 37 | 103 |
| 2022 June | 31 | 28 | 59 |
| 2022 May | 40 | 38 | 78 |
| 2022 April | 30 | 33 | 63 |
| 2022 March | 35 | 45 | 80 |
| 2022 February | 23 | 30 | 53 |
| 2022 January | 33 | 36 | 69 |
| 2021 December | 26 | 49 | 75 |
| 2021 November | 43 | 49 | 92 |
| 2021 October | 38 | 51 | 89 |
| 2021 September | 31 | 33 | 64 |
| 2021 August | 36 | 19 | 55 |
| 2021 July | 17 | 26 | 43 |
| 2021 June | 30 | 18 | 48 |
| 2021 May | 26 | 21 | 47 |
| 2021 April | 33 | 63 | 96 |
| 2021 March | 48 | 22 | 70 |
| 2021 February | 44 | 25 | 69 |
| 2021 January | 35 | 12 | 47 |
| 2020 December | 28 | 17 | 45 |
| 2020 November | 22 | 21 | 43 |
| 2020 October | 40 | 12 | 52 |
| 2020 September | 33 | 14 | 47 |
| 2020 August | 21 | 16 | 37 |
| 2020 July | 28 | 13 | 41 |
| 2020 June | 32 | 24 | 56 |
| 2020 May | 23 | 22 | 45 |
| 2020 April | 21 | 16 | 37 |
| 2020 March | 30 | 20 | 50 |
| 2020 February | 2 | 3 | 5 |
| 2020 January | 0 | 3 | 3 |
| 2019 December | 2 | 4 | 6 |
| 2019 September | 4 | 2 | 6 |
| 2019 July | 0 | 7 | 7 |
| 2019 June | 2 | 3 | 5 |
| 2019 May | 0 | 16 | 16 |
| 2019 April | 0 | 9 | 9 |
| 2019 March | 2 | 3 | 5 |
| 2019 February | 0 | 2 | 2 |
| 2019 January | 4 | 0 | 4 |
| 2018 December | 4 | 2 | 6 |
| 2018 November | 2 | 2 | 4 |
| 2018 October | 3 | 2 | 5 |
| 2018 September | 10 | 0 | 10 |
| 2018 February | 27 | 2 | 29 |
| 2018 January | 32 | 5 | 37 |
| 2017 December | 44 | 8 | 52 |
| 2017 November | 26 | 5 | 31 |
| 2017 October | 35 | 6 | 41 |
| 2017 September | 31 | 7 | 38 |
| 2017 August | 35 | 5 | 40 |
| 2017 July | 29 | 4 | 33 |
| 2017 June | 51 | 10 | 61 |
| 2017 May | 47 | 4 | 51 |
| 2017 April | 28 | 9 | 37 |
| 2017 March | 21 | 24 | 45 |
| 2017 February | 22 | 9 | 31 |
| 2017 January | 25 | 8 | 33 |
| 2016 December | 51 | 11 | 62 |
| 2016 November | 53 | 21 | 74 |
| 2016 October | 38 | 19 | 57 |
| 2016 September | 0 | 3 | 3 |
| 2016 July | 1 | 1 | 2 |
| 2016 June | 3 | 2 | 5 |
| 2016 May | 2 | 3 | 5 |
| 2016 April | 0 | 13 | 13 |
| 2016 February | 0 | 1 | 1 |
| 2016 January | 0 | 2 | 2 |
| 2015 December | 0 | 3 | 3 |
| 2015 November | 0 | 4 | 4 |
Show all
