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Multiple painless papulonodules in a 3-year-old girl: type A lymphomatoid papulosis
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Caso clínico</span>" "titulo" => "Múltiplos papulonódulos indolores em menina de três anos" "tienePdf" => "pt" "tieneTextoCompleto" => "pt" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "689" "paginaFinal" => "690" ] ] "contieneTextoCompleto" => array:1 [ "pt" => true ] "contienePdf" => array:1 [ "pt" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figura 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1846 "Ancho" => 1508 "Tamanyo" => 196827 ] ] "descripcion" => array:1 [ "pt" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Pápulas e nódulos eritemato‐violáceos com discreta descamação nos membros inferiores, um deles com ulceração espontânea (coxa direita).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Nuno Gomes, Ana Nogueira, Roberto Silva, Filomena 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"LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "en" => array:10 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Case Letter</span>" "titulo" => "Eruptive collagenoma in a juvenile patient with Down syndrome" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "687" "paginaFinal" => "688" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1136 "Ancho" => 1508 "Tamanyo" => 102504 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Small oval, slightly reddish or skin-colored papules scattered on the back (arrow).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Yasunobu Kato, Toshiyuki Yamamoto" "autores" => array:2 [ 0 => array:2 [ "nombre" => "Yasunobu" "apellidos" => "Kato" ] 1 => array:2 [ "nombre" => "Toshiyuki" "apellidos" => "Yamamoto" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0365059622001234?idApp=UINPBA00008Z" "url" => "/03650596/0000009700000005/v1_202208290604/S0365059622001234/v1_202208290604/en/main.assets" ] "en" => array:17 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Case Letter</span>" "titulo" => "Multiple painless papulonodules in a 3-year-old girl: type A lymphomatoid papulosis" "tieneTextoCompleto" => true "saludo" => "Dear Editor," "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "689" "paginaFinal" => "690" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Nuno Gomes, Ana Nogueira, Roberto Silva, Filomena Azevedo" "autores" => array:4 [ 0 => array:4 [ "nombre" => "Nuno" "apellidos" => "Gomes" "email" => array:1 [ 0 => "nunompretogomes@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" => "Ana" "apellidos" => "Nogueira" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Roberto" "apellidos" => "Silva" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "Filomena" "apellidos" => "Azevedo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Department of Dermatovenereology of Centro Hospitalar, Universitário de São João EPE, Porto, Portugal" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Department of Pathology of Centro Hospitalar, Universitário de São João EPE, Porto, Portugal" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1133 "Ancho" => 1508 "Tamanyo" => 561876 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0015" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Multifocal dermal infiltrate of small lymphocytes, some plasma cells and histiocytes, as well as several lymphoid cells with amphophilic cytoplasm and big nucleus with prominent nucleolus (Hematoxylin & eosin, ×100).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 3-year-old girl, otherwise healthy, presented with a dermatosis characterized by asymptomatic erythematoviolaceous papules and nodules with slight desquamation on the trunk and lower limbs, one of them with spontaneous ulceration, evolving for 3 months (<a class="elsevierStyleCrossRefs" href="#fig0005">Figs. 1 and 2</a>). She was in a good general condition and no lymph nodes were palpable. Her mother denied any previous infectious episodes. She had been applying a methylprednisolone aceponate cream for the previous 2 weeks without improvement. She had no significant past medical or family history. A skin biopsy of the lower right limb was performed (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). A diagnosis of lymphomatoid papulosis was made.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Histopathology showed a multifocal dermal infiltrate of small lymphocytes, some plasma cells, and histiocytes, as well as several lymphoid cells with amphophilic cytoplasm, a big nucleus with the prominent nucleolus, and positivity to CD30, consistent with a type A lymphomatoid papulosis (LyP). The patient was treated with clobetasol propionate 0.5 mg/g ointment twice daily with progressive clearance of the lesions and has been symptom-free for the last four years.</p><p id="par0015" class="elsevierStylePara elsevierViewall">LyP is a rare lymphoproliferative disorder that is included in the spectrum of primary cutaneous CD30-positive T-cell lymphoproliferative disorders by the World Health Organization classification of lymphoid tissue tumors.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> It slightly affects more men than women and usually presents with papulonodular lesions on the limbs and trunk, sometimes with necrosis, developing in crops.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,3</span></a> Pruritus is a common feature.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">LyP is a recurrent disease, with a typical waning and waxing course of self-healing lesions.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Lyp is seen most commonly in adults, although it has been suggested that the spontaneous resolution of lesions and their limited number in pediatric age may underestimate its frequency.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> In fact, the clinical presentation and course of LyP in childhood differs very little from the adult form.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Albeit being rare among pediatric patients, LyP is one of the two most prevalent cutaneous lymphoproliferative diseases at this age, after mycosis fungoides.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Over the years, the histopathological classification of LyP has been a changing scenario. Currently, five classically recognized histopathological subtypes have been defined, which vary based on the predominant cell type and tropism: type A (mixed cellular), type B (epidermotropic), type C (cohesive infiltrate), type D (epidermotropism), and type E (angiocentric and angiodestructive).<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3,4</span></a> The histopathological characterization has no prognostic value nor guides the treatment algorithm. The most common pathologic subtype of LyP is type A (also observed in the present study’s patient), which is characterized by a wedge-shaped dense dermal perivascular lymphoid infiltrate with large atypical CD30-positive cells with Reed-Sternberg appearance.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,5</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The differential diagnosis of LyP includes both forms of pityriasis lichenoides (pityriasis lichenoides et varioliformis acuta ‒ PLEVA ‒ and pityriasis lichenoides chronica ‒ PLC), primary cutaneous anaplastic large cell lymphoma (ALCL), a papular variant of mycosis fungoides and arthropod bites. More remotely, but mainly in childhood, other diagnoses such as scabies, pityriasis rosea, guttate psoriasis, and eczema should be considered.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a> Some clinical tips may help in differentiating these diseases. For instance, as opposed to LyP, primary cutaneous ALCL presents usually as a solitary lesion and PLEVA presents with smaller lesions that usually do not show a waxing and waning nature and heal without scarring.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">In the absence of molecular or immunohistochemical criteria to predict LyP evolution, the clinicopathological correlation and long-term follow-up are indispensable. In fact, although LyP has classically a benign and indolent clinical course, some cases progress to other types of lymphoma, such as primary cutaneous ALCL, mycosis fungoides, or Hodgkin disease.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,4</span></a> Fortunately, in pediatric patients, progression to lymphoma is exceptional.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Moreover, some factors such as association with viral infections, atopic dermatitis, skin eosinophilic infiltration, and spontaneous complete remission strongly suggest that pediatric LyP could be considered a reactional disease rather than a neoplastic disorder.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The long-term prognosis of pediatric LyP is generally considered to be good, with a 10-year disease-related survival rate of 100%.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a> First-line treatment generally comprises therapeutic abstention or skin-directed modalities such as topical steroids.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3</span></a> Methotrexate and phototherapy are also commonly prescribed.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> There is no decreased risk of lymphoma in patients submitted to treatment, whereby the “wait and see” approach is also often applied.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,5</span></a></p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Financial support</span><p id="par0045" class="elsevierStylePara elsevierViewall">None declared.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Authors’ contributions</span><p id="par0050" class="elsevierStylePara elsevierViewall">Nuno Gomes: Conception and design of the study, or acquisition of data, or analysis and interpretation of data, drafting the article or revising it critically for important intellectual content, final approval of the version to be submitted.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Ana Nogueira: Conception and design of the study, or acquisition of data, or analysis and interpretation of data, drafting the article or revising it critically for important intellectual content, final approval of the version to be submitted</p><p id="par0060" class="elsevierStylePara elsevierViewall">Roberto Silva: Conception and design of the study, or acquisition of data, or analysis and interpretation of data, drafting the article or revising it critically for important intellectual content, final approval of the version to be submitted</p><p id="par0065" class="elsevierStylePara elsevierViewall">Filomena Azevedo: Conception and design of the study, or acquisition of data, or analysis and interpretation of data, drafting the article or revising it critically for important intellectual content, final approval of the version to be submitted</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">None declared.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Financial support" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Authors’ contributions" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Conflicts of interest" ] 3 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2020-07-24" "fechaAceptado" => "2020-11-08" "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Study conducted at the Centro Hospitalar Universitário de São João EPE, Porto, Portugal.</p>" ] ] "multimedia" => array:3 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1340 "Ancho" => 1508 "Tamanyo" => 158338 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Erythematoviolaceous papules and nodules with slight desquamation on the lower limbs, one of them with spontaneous ulceration (right thigh).</p>" ] ] 1 => array:8 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1742 "Ancho" => 1508 "Tamanyo" => 169009 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Erythematoviolaceous papules and nodules with slight desquamation on the abdomen and lower limbs.</p>" ] ] 2 => array:8 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1133 "Ancho" => 1508 "Tamanyo" => 561876 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0015" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Multifocal dermal infiltrate of small lymphocytes, some plasma cells and histiocytes, as well as several lymphoid cells with amphophilic cytoplasm and big nucleus with prominent nucleolus (Hematoxylin & eosin, ×100).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Lymphomatoid papulosis: an update and review" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "S.A. 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| Ano/Mês | Html | Total | |
|---|---|---|---|
| 2024 Novembro | 18 | 8 | 26 |
| 2024 Outubro | 134 | 83 | 217 |
| 2024 Setembro | 173 | 83 | 256 |
| 2024 Agosto | 160 | 126 | 286 |
| 2024 Julho | 153 | 118 | 271 |
| 2024 Junho | 109 | 67 | 176 |
| 2024 Maio | 119 | 65 | 184 |
| 2024 Abril | 126 | 85 | 211 |
| 2024 Março | 99 | 67 | 166 |
| 2024 Fevereiro | 90 | 84 | 174 |
| 2024 Janeiro | 81 | 49 | 130 |
| 2023 Dezembro | 77 | 56 | 133 |
| 2023 Novembro | 79 | 85 | 164 |
| 2023 Outubro | 96 | 75 | 171 |
| 2023 Setembro | 172 | 82 | 254 |
| 2023 Agosto | 48 | 31 | 79 |
| 2023 Julho | 84 | 32 | 116 |
| 2023 Junho | 80 | 47 | 127 |
| 2023 Maio | 49 | 31 | 80 |
| 2023 Abril | 39 | 21 | 60 |
| 2023 Março | 53 | 47 | 100 |
| 2023 Fevereiro | 36 | 44 | 80 |
| 2023 Janeiro | 44 | 33 | 77 |
| 2022 Dezembro | 78 | 51 | 129 |
| 2022 Novembro | 92 | 63 | 155 |
| 2022 Outubro | 90 | 66 | 156 |
| 2022 Setembro | 111 | 94 | 205 |
| 2022 Agosto | 61 | 56 | 117 |
| 2022 Julho | 28 | 33 | 61 |
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