array:24 [
  "pii" => "S0001731023005379"
  "issn" => "00017310"
  "doi" => "10.1016/j.ad.2023.07.005"
  "estado" => "S300"
  "fechaPublicacion" => "2023-09-01"
  "aid" => "3582"
  "copyright" => "AEDV"
  "copyrightAnyo" => "2022"
  "documento" => "simple-article"
  "crossmark" => 1
  "subdocumento" => "crp"
  "cita" => "Actas Dermosifiliogr. 2023;114:T729-T730"
  "abierto" => array:3 [
    "ES" => true
    "ES2" => true
    "LATM" => true
  ]
  "gratuito" => true
  "lecturas" => array:1 [
    "total" => 0
  ]
  "itemSiguiente" => array:18 [
    "pii" => "S0001731023003332"
    "issn" => "00017310"
    "doi" => "10.1016/j.ad.2022.07.035"
    "estado" => "S300"
    "fechaPublicacion" => "2023-09-01"
    "aid" => "3483"
    "copyright" => "AEDV"
    "documento" => "simple-article"
    "crossmark" => 1
    "subdocumento" => "crp"
    "cita" => "Actas Dermosifiliogr. 2023;114:731-2"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:1 [
      "total" => 0
    ]
    "es" => array:11 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Casos para el diagn&#243;stico</span>"
      "titulo" => "N&#243;dulos subcut&#225;neos y eritema palpebral en paciente con compromiso pulmonar"
      "tienePdf" => "es"
      "tieneTextoCompleto" => "es"
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "731"
          "paginaFinal" => "732"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "en" => array:1 [
          "titulo" => "Subcutaneous Nodules and Eyelid Erythema in a Patient With Pulmonary Involvement"
        ]
      ]
      "contieneTextoCompleto" => array:1 [
        "es" => true
      ]
      "contienePdf" => array:1 [
        "es" => true
      ]
      "resumenGrafico" => array:2 [
        "original" => 0
        "multimedia" => array:6 [
          "identificador" => "fig0005"
          "etiqueta" => "Figura 1"
          "tipo" => "MULTIMEDIAFIGURA"
          "mostrarFloat" => true
          "mostrarDisplay" => false
          "figura" => array:1 [
            0 => array:4 [
              "imagen" => "gr1.jpeg"
              "Alto" => 432
              "Ancho" => 1328
              "Tamanyo" => 112131
            ]
          ]
        ]
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "A&#46; Gil-Villalba, D&#46; Moyano-Bueno, R&#46; Ruiz-Villaverde"
          "autores" => array:3 [
            0 => array:2 [
              "nombre" => "A&#46;"
              "apellidos" => "Gil-Villalba"
            ]
            1 => array:2 [
              "nombre" => "D&#46;"
              "apellidos" => "Moyano-Bueno"
            ]
            2 => array:2 [
              "nombre" => "R&#46;"
              "apellidos" => "Ruiz-Villaverde"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "es"
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0001731023003332?idApp=UINPBA000044"
    "url" => "/00017310/0000011400000008/v2_202310161122/S0001731023003332/v2_202310161122/es/main.assets"
  ]
  "itemAnterior" => array:18 [
    "pii" => "S0001731022010420"
    "issn" => "00017310"
    "doi" => "10.1016/j.ad.2022.03.028"
    "estado" => "S300"
    "fechaPublicacion" => "2023-09-01"
    "aid" => "3329"
    "copyright" => "AEDV"
    "documento" => "simple-article"
    "crossmark" => 1
    "subdocumento" => "crp"
    "cita" => "Actas Dermosifiliogr. 2023;114:729-30"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:1 [
      "total" => 0
    ]
    "es" => array:11 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Casos para el diagn&#243;stico</span>"
      "titulo" => "Hemangiomas glomeruloides de reciente aparici&#243;n"
      "tienePdf" => "es"
      "tieneTextoCompleto" => "es"
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "729"
          "paginaFinal" => "730"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "en" => array:1 [
          "titulo" => "Glomeruloid Hemangiomas of Recent Appearance"
        ]
      ]
      "contieneTextoCompleto" => array:1 [
        "es" => true
      ]
      "contienePdf" => array:1 [
        "es" => true
      ]
      "resumenGrafico" => array:2 [
        "original" => 0
        "multimedia" => array:7 [
          "identificador" => "fig0010"
          "etiqueta" => "Figura 2"
          "tipo" => "MULTIMEDIAFIGURA"
          "mostrarFloat" => true
          "mostrarDisplay" => false
          "figura" => array:1 [
            0 => array:4 [
              "imagen" => "gr2.jpeg"
              "Alto" => 761
              "Ancho" => 950
              "Tamanyo" => 230107
            ]
          ]
          "descripcion" => array:1 [
            "es" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A&#41;<span class="elsevierStyleHsp" style=""></span>Hematoxilina-eosina &#40;&#215;5&#41;&#46; B&#41;<span class="elsevierStyleHsp" style=""></span>Hematoxilina-eosina &#40;&#215;10&#41;&#46; C y D&#41;<span class="elsevierStyleHsp" style=""></span>Hematoxilina-eosina &#40;&#215;40&#41;&#46;</p>"
          ]
        ]
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "J&#46;F&#46; Orts Paco, E&#46; Cutillas Marco, L&#46; Lorente Gea"
          "autores" => array:3 [
            0 => array:2 [
              "nombre" => "J&#46;F&#46;"
              "apellidos" => "Orts Paco"
            ]
            1 => array:2 [
              "nombre" => "E&#46;"
              "apellidos" => "Cutillas Marco"
            ]
            2 => array:2 [
              "nombre" => "L&#46;"
              "apellidos" => "Lorente Gea"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "es"
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0001731022010420?idApp=UINPBA000044"
    "url" => "/00017310/0000011400000008/v2_202310161122/S0001731022010420/v2_202310161122/es/main.assets"
  ]
  "asociados" => array:1 [
    0 => array:18 [
      "pii" => "S0001731022010420"
      "issn" => "00017310"
      "doi" => "10.1016/j.ad.2022.03.028"
      "estado" => "S300"
      "fechaPublicacion" => "2023-09-01"
      "aid" => "3329"
      "copyright" => "AEDV"
      "documento" => "simple-article"
      "crossmark" => 1
      "subdocumento" => "crp"
      "cita" => "Actas Dermosifiliogr. 2023;114:729-30"
      "abierto" => array:3 [
        "ES" => true
        "ES2" => true
        "LATM" => true
      ]
      "gratuito" => true
      "lecturas" => array:1 [
        "total" => 0
      ]
      "es" => array:11 [
        "idiomaDefecto" => true
        "cabecera" => "<span class="elsevierStyleTextfn">Casos para el diagn&#243;stico</span>"
        "titulo" => "Hemangiomas glomeruloides de reciente aparici&#243;n"
        "tienePdf" => "es"
        "tieneTextoCompleto" => "es"
        "paginas" => array:1 [
          0 => array:2 [
            "paginaInicial" => "729"
            "paginaFinal" => "730"
          ]
        ]
        "titulosAlternativos" => array:1 [
          "en" => array:1 [
            "titulo" => "Glomeruloid Hemangiomas of Recent Appearance"
          ]
        ]
        "contieneTextoCompleto" => array:1 [
          "es" => true
        ]
        "contienePdf" => array:1 [
          "es" => true
        ]
        "resumenGrafico" => array:2 [
          "original" => 0
          "multimedia" => array:7 [
            "identificador" => "fig0010"
            "etiqueta" => "Figura 2"
            "tipo" => "MULTIMEDIAFIGURA"
            "mostrarFloat" => true
            "mostrarDisplay" => false
            "figura" => array:1 [
              0 => array:4 [
                "imagen" => "gr2.jpeg"
                "Alto" => 761
                "Ancho" => 950
                "Tamanyo" => 230107
              ]
            ]
            "descripcion" => array:1 [
              "es" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A&#41;<span class="elsevierStyleHsp" style=""></span>Hematoxilina-eosina &#40;&#215;5&#41;&#46; B&#41;<span class="elsevierStyleHsp" style=""></span>Hematoxilina-eosina &#40;&#215;10&#41;&#46; C y D&#41;<span class="elsevierStyleHsp" style=""></span>Hematoxilina-eosina &#40;&#215;40&#41;&#46;</p>"
            ]
          ]
        ]
        "autores" => array:1 [
          0 => array:2 [
            "autoresLista" => "J&#46;F&#46; Orts Paco, E&#46; Cutillas Marco, L&#46; Lorente Gea"
            "autores" => array:3 [
              0 => array:2 [
                "nombre" => "J&#46;F&#46;"
                "apellidos" => "Orts Paco"
              ]
              1 => array:2 [
                "nombre" => "E&#46;"
                "apellidos" => "Cutillas Marco"
              ]
              2 => array:2 [
                "nombre" => "L&#46;"
                "apellidos" => "Lorente Gea"
              ]
            ]
          ]
        ]
      ]
      "idiomaDefecto" => "es"
      "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0001731022010420?idApp=UINPBA000044"
      "url" => "/00017310/0000011400000008/v2_202310161122/S0001731022010420/v2_202310161122/es/main.assets"
    ]
  ]
  "en" => array:14 [
    "idiomaDefecto" => true
    "cabecera" => "<span class="elsevierStyleTextfn">Case for Diagnosis</span>"
    "titulo" => " Glomeruloid Hemangiomas of Recent Appearance"
    "tieneTextoCompleto" => true
    "paginas" => array:1 [
      0 => array:2 [
        "paginaInicial" => "T729"
        "paginaFinal" => "T730"
      ]
    ]
    "autores" => array:1 [
      0 => array:4 [
        "autoresLista" => "J&#46;F&#46; Orts Paco, E&#46; Cutillas Marco, L&#46; Lorente Gea"
        "autores" => array:3 [
          0 => array:4 [
            "nombre" => "J&#46;F&#46;"
            "apellidos" => "Orts Paco"
            "email" => array:1 [
              0 => "jose.orts.95@gmail.com"
            ]
            "referencia" => array:2 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">a</span>"
                "identificador" => "aff0005"
              ]
              1 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">&#42;</span>"
                "identificador" => "cor0005"
              ]
            ]
          ]
          1 => array:3 [
            "nombre" => "E&#46;"
            "apellidos" => "Cutillas Marco"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">a</span>"
                "identificador" => "aff0005"
              ]
            ]
          ]
          2 => array:3 [
            "nombre" => "L&#46;"
            "apellidos" => "Lorente Gea"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">b</span>"
                "identificador" => "aff0010"
              ]
            ]
          ]
        ]
        "afiliaciones" => array:2 [
          0 => array:3 [
            "entidad" => "Servicio de Dermatolog&#237;a&#44; Hospital General Universitario Reina Sof&#237;a&#44; Murcia&#44; Spain"
            "etiqueta" => "a"
            "identificador" => "aff0005"
          ]
          1 => array:3 [
            "entidad" => "Servicio de Anatom&#237;a Patol&#243;gica&#44; Hospital General Universitario Reina Sof&#237;a&#44; Murcia&#44; Spain"
            "etiqueta" => "b"
            "identificador" => "aff0010"
          ]
        ]
        "correspondencia" => array:1 [
          0 => array:3 [
            "identificador" => "cor0005"
            "etiqueta" => "&#8270;"
            "correspondencia" => "Corresponding author&#46;"
          ]
        ]
      ]
    ]
    "titulosAlternativos" => array:1 [
      "es" => array:1 [
        "titulo" => "Hemangiomas glomeruloides de reciente aparici&#243;n"
      ]
    ]
    "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" => 761
            "Ancho" => 950
            "Tamanyo" => 230107
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Hematoxylin-eosin&#44; &#215;5&#46; &#40;B&#41; Hematoxylin-eosin&#44; &#215;10&#46; &#40;C and D&#41; Hematoxylin-eosin&#44; &#215;40&#46;</p>"
        ]
      ]
    ]
    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">The image corresponds to a 38-year-old man who was admitted to the neurology department with a 3-month history of weakness in both legs and difficulty walking &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Physical examination revealed leg weakness&#44; tandem gait difficulty&#44; and universal areflexia&#46; During admission&#44; the patient was observed to have hepatosplenomegaly&#44; multiple enlarged lymph nodes&#44; and thrombocytosis&#46; The neurophysiological study revealed predominantly demyelinating sensorimotor polyneuropathy&#46; The initial diagnosis was chronic inflammatory demyelinating polyneuropathy&#44; and the patient received treatment with corticosteroids and intravenous immunoglobulin boluses&#46; Three months later&#44; owing to a loss of visual acuity&#44; he was assessed in ophthalmology&#44; where he was diagnosed with incipient bilateral papilledema&#46; He also underwent lumbar puncture&#44; with normal opening pressure and markedly increased cerebrospinal fluid proteins&#46; In addition&#44; he had subclinical hypothyroidism and slightly increased prolactin values&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Five recent lesions were observed on the skin&#46; Examination revealed red dome-shaped papules that were clinically and dermoscopically compatible with cherry angiomas &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Examination of a punch biopsy specimen from the largest lesion indicated glomeruloid hemangiomas &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">What is your diagnosis&#63;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnosis</span><p id="par0025" class="elsevierStylePara elsevierViewall">POEMS syndrome&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical Course and Treatment</span><p id="par0030" class="elsevierStylePara elsevierViewall">Extension of the diagnostic study revealed increased vascular endothelial growth factor &#40;VEGF&#41; and absence of clonal plasma cells&#44; leading to a diagnosis of atypical&#44; or incomplete&#44; POEMS syndrome&#46; Four months later&#44; analysis revealed the presence of a low-intensity monoclonal immunoglobulin G &#954; component in blood&#44; with markedly increased VEGF values &#40;2407<span class="elsevierStyleHsp" style=""></span>pg&#47;mL&#41;&#46; Therefore&#44; a definitive diagnosis of POEMS syndrome was made based on the presence of the 2 mandatory components &#40;polyneuropathy and a monoclonal component&#41;&#44; a major component &#40;increased VEGF&#41;&#44; and 5 minor components &#40;hepatosplenomegaly&#44; endocrinopathy&#44; skin lesions&#44; papilledema&#44; and thrombocytosis&#41;&#46; Consequently&#44; the patient underwent autologous stem cell transplantation&#44; with a favorable initial response&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comment</span><p id="par0035" class="elsevierStylePara elsevierViewall">POEMS syndrome is a rare paraneoplastic syndrome caused by underlying monoclonal plasma cell dyscrasia&#46; The acronym was coined by Bardwick in 1980 to refer to several of the characteristics of the syndrome&#58; polyradiculoneuropathy&#44; organomegaly&#44; endocrinopathy&#44; monoclonal plasma cell dyscrasia&#44; and skin changes&#46; To date&#44; VEGF is the cytokine that best correlates with disease activity&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> The cutoff values for POEMS syndrome are 200<span class="elsevierStyleHsp" style=""></span>pg&#47;mL in plasma &#40;specificity of 95&#37; and sensitivity of 68&#37;&#41; and 1920<span class="elsevierStyleHsp" style=""></span>pg&#47;mL in serum &#40;specificity of 98&#37; and sensitivity of 73&#37;&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;2</span></a> Diagnosis is based on the criteria of Dispenzieri&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> which require 2 mandatory criteria to be met&#44; namely&#44; 1 major and 1 minor&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Skin manifestations affect 70&#37;&#8211;90&#37; of patients and include hyperpigmentation&#44; acrocyanosis&#44; facial plethora&#44; hemangiomas&#44; hypertrichosis&#44; and scleroderma-like skin changes&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;3</span></a> Glomeruloid hemangioma was first described by Chan et al&#46; in 1990&#44; and while it is uncommon&#44; it constitutes the most specific skin manifestation of POEMS syndrome&#44; with a strong association between both&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> It is believed to be a proliferative reaction to angiogenic stimuli &#40;VEGF&#44; interleukin 1&#44; and tumor necrosis factor&#41; and usually appears abruptly as multiple violaceous dome-shaped papules with a vascular appearance on the trunk and proximal area of the extremities&#46; Sudden onset over a few days or weeks helps to establish an association between glomeruloid hemangioma and POEMS syndrome&#44; since a slower onset may be observed in patients with no associated symptoms&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> Furthermore&#44; it is important to stress that glomeruloid hemangiomas can precede full development of the disease by some years&#44; thus providing an early sign of POEMS syndrome&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Atypical&#44; or incomplete&#44; POEMS syndrome<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#44;6</span></a> is characterized by the absence of one of the mandatory criteria&#46; This condition requires close follow-up&#44; since progression to the complete form has been reported&#44; as in the case we present&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Treatment ranges from radiotherapy to systemic approaches similar to those used in multiple myeloma&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflicts of Interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
    "textoCompletoSecciones" => array:1 [
      "secciones" => array:8 [
        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:2 [
          "identificador" => "sec0035"
          "titulo" => "Conflicts of Interest"
        ]
        7 => array:1 [
          "titulo" => "References"
        ]
      ]
    ]
    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "multimedia" => array:2 [
      0 => array:6 [
        "identificador" => "fig0005"
        "etiqueta" => "Figure 1"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
            "imagen" => "gr1.jpeg"
            "Alto" => 492
            "Ancho" => 1005
            "Tamanyo" => 75961
          ]
        ]
      ]
      1 => array:7 [
        "identificador" => "fig0010"
        "etiqueta" => "Figure 2"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
            "imagen" => "gr2.jpeg"
            "Alto" => 761
            "Ancho" => 950
            "Tamanyo" => 230107
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Hematoxylin-eosin&#44; &#215;5&#46; &#40;B&#41; Hematoxylin-eosin&#44; &#215;10&#46; &#40;C and D&#41; Hematoxylin-eosin&#44; &#215;40&#46;</p>"
        ]
      ]
    ]
    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0015"
          "bibliografiaReferencia" => array:6 [
            0 => array:3 [
              "identificador" => "bib0035"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "POEMS syndrome&#58; 2019 update on diagnosis&#44; risk-stratification&#44; and management"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "A&#46; Dispenzieri"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1002/ajh.25495"
                      "Revista" => array:6 [
                        "tituloSerie" => "Am J Hematol"
                        "fecha" => "2019"
                        "volumen" => "94"
                        "paginaInicial" => "812"
                        "paginaFinal" => "827"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/31012139"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0040"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "A&#46; D&#39;Souza"
                            1 => "S&#46;R&#46; Hayman"
                            2 => "F&#46; Buadi"
                            3 => "M&#46; Mauermann"
                            4 => "M&#46;Q&#46; Lacy"
                            5 => "M&#46;A&#46; Gertz"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1182/blood-2011-06-362392"
                      "Revista" => array:6 [
                        "tituloSerie" => "Blood"
                        "fecha" => "2011"
                        "volumen" => "118"
                        "paginaInicial" => "4663"
                        "paginaFinal" => "4665"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21881050"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0045"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Hemangiomas glomeruloides y s&#237;ndrome POEMS"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "I&#46; Hern&#225;ndez Arag&#252;&#233;s"
                            1 => "A&#46; Pulido P&#233;rez"
                            2 => "C&#46; Ciudad Blanco"
                            3 => "V&#46; Parra Blanco"
                            4 => "R&#46; Su&#225;rez Fern&#225;ndez"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.ad.2015.10.020"
                      "Revista" => array:6 [
                        "tituloSerie" => "Actas Dermosifiliogr"
                        "fecha" => "2017"
                        "volumen" => "108"
                        "paginaInicial" => "e15"
                        "paginaFinal" => "e19"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27208909"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib0050"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "POEMS syndrome&#58; are current diagnostic criteria too exclusive&#63;"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "Y&#46; Charli-Joseph"
                            1 => "M&#46; Fern&#225;ndez-S&#225;nchez"
                            2 => "M&#46; Saeb-Lima"
                            3 => "R&#46; Orozco-Topete"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.jaad.2010.06.046"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Am Acad Dermatol"
                        "fecha" => "2011"
                        "volumen" => "65"
                        "paginaInicial" => "415"
                        "paginaFinal" => "417"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21501892"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            4 => array:3 [
              "identificador" => "bib0055"
              "etiqueta" => "5"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Glomeruloid hemangioma as a marker for the early diagnosis of POEMS syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "J&#46;Y&#46; Lee"
                            1 => "J&#46;K&#46; Choi"
                            2 => "J&#46;W&#46; Ha"
                            3 => "S&#46;E&#46; Park"
                            4 => "C&#46;W&#46; Kim"
                            5 => "S&#46;S&#46; Kim"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Ann Dermatol"
                        "fecha" => "2017"
                        "volumen" => "29"
                        "paginaInicial" => "249"
                        "paginaFinal" => "251"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            5 => array:3 [
              "identificador" => "bib0060"
              "etiqueta" => "6"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Clinical characteristics and the long-term outcome of patients with atypical POEMS syndrome variant with undetectable monoclonal gammopathy"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "T&#46; He"
                            1 => "A&#46; Zhao"
                            2 => "H&#46; Zhao"
                            3 => "H&#46; Cai"
                            4 => "J&#46; Feng"
                            5 => "L&#46; Zhang"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1007/s00277-018-03589-4"
                      "Revista" => array:6 [
                        "tituloSerie" => "Ann Hematol"
                        "fecha" => "2019"
                        "volumen" => "98"
                        "paginaInicial" => "735"
                        "paginaFinal" => "743"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/30612232"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
          ]
        ]
      ]
    ]
  ]
  "idiomaDefecto" => "en"
  "url" => "/00017310/0000011400000008/v2_202310161122/S0001731023005379/v2_202310161122/en/main.assets"
  "Apartado" => array:4 [
    "identificador" => "6161"
    "tipo" => "SECCION"
    "es" => array:2 [
      "titulo" => "Casos para el diagn&#243;stico"
      "idiomaDefecto" => true
    ]
    "idiomaDefecto" => "es"
  ]
  "PDF" => "https://static.elsevier.es/multimedia/00017310/0000011400000008/v2_202310161122/S0001731023005379/v2_202310161122/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/"
  "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0001731023005379?idApp=UINPBA000044"
]
Compartir
Información de la revista
Vol. 114. Núm. 8.
Páginas T729-T730 (septiembre 2023)
Compartir
Compartir
Descargar PDF
Más opciones de artículo
Visitas
2301
Vol. 114. Núm. 8.
Páginas T729-T730 (septiembre 2023)
Case for Diagnosis
Acceso a texto completo
Glomeruloid Hemangiomas of Recent Appearance
Hemangiomas glomeruloides de reciente aparición
Visitas
2301
J.F. Orts Pacoa,
Autor para correspondencia
jose.orts.95@gmail.com

Corresponding author.
, E. Cutillas Marcoa, L. Lorente Geab
a Servicio de Dermatología, Hospital General Universitario Reina Sofía, Murcia, Spain
b Servicio de Anatomía Patológica, Hospital General Universitario Reina Sofía, Murcia, Spain
Contenido relacionado
Actas Dermosifiliogr. 2023;114:729-3010.1016/j.ad.2022.03.028
J.F. Orts Paco, E. Cutillas Marco, L. Lorente Gea
Este artículo ha recibido
Información del artículo
Texto completo
Bibliografía
Descargar PDF
Estadísticas
Figuras (2)
Texto completo
Medical History

The image corresponds to a 38-year-old man who was admitted to the neurology department with a 3-month history of weakness in both legs and difficulty walking (Fig. 1). Physical examination revealed leg weakness, tandem gait difficulty, and universal areflexia. During admission, the patient was observed to have hepatosplenomegaly, multiple enlarged lymph nodes, and thrombocytosis. The neurophysiological study revealed predominantly demyelinating sensorimotor polyneuropathy. The initial diagnosis was chronic inflammatory demyelinating polyneuropathy, and the patient received treatment with corticosteroids and intravenous immunoglobulin boluses. Three months later, owing to a loss of visual acuity, he was assessed in ophthalmology, where he was diagnosed with incipient bilateral papilledema. He also underwent lumbar puncture, with normal opening pressure and markedly increased cerebrospinal fluid proteins. In addition, he had subclinical hypothyroidism and slightly increased prolactin values.

Figure 1
(0.07MB).
Physical Examination

Five recent lesions were observed on the skin. Examination revealed red dome-shaped papules that were clinically and dermoscopically compatible with cherry angiomas (Fig. 1).

Histopathology

Examination of a punch biopsy specimen from the largest lesion indicated glomeruloid hemangiomas (Fig. 2).

Figure 2.

(A) Hematoxylin-eosin, ×5. (B) Hematoxylin-eosin, ×10. (C and D) Hematoxylin-eosin, ×40.

(0.22MB).

What is your diagnosis?

Diagnosis

POEMS syndrome.

Clinical Course and Treatment

Extension of the diagnostic study revealed increased vascular endothelial growth factor (VEGF) and absence of clonal plasma cells, leading to a diagnosis of atypical, or incomplete, POEMS syndrome. Four months later, analysis revealed the presence of a low-intensity monoclonal immunoglobulin G κ component in blood, with markedly increased VEGF values (2407pg/mL). Therefore, a definitive diagnosis of POEMS syndrome was made based on the presence of the 2 mandatory components (polyneuropathy and a monoclonal component), a major component (increased VEGF), and 5 minor components (hepatosplenomegaly, endocrinopathy, skin lesions, papilledema, and thrombocytosis). Consequently, the patient underwent autologous stem cell transplantation, with a favorable initial response.

Comment

POEMS syndrome is a rare paraneoplastic syndrome caused by underlying monoclonal plasma cell dyscrasia. The acronym was coined by Bardwick in 1980 to refer to several of the characteristics of the syndrome: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell dyscrasia, and skin changes. To date, VEGF is the cytokine that best correlates with disease activity.1 The cutoff values for POEMS syndrome are 200pg/mL in plasma (specificity of 95% and sensitivity of 68%) and 1920pg/mL in serum (specificity of 98% and sensitivity of 73%).1,2 Diagnosis is based on the criteria of Dispenzieri,1 which require 2 mandatory criteria to be met, namely, 1 major and 1 minor.

Skin manifestations affect 70%–90% of patients and include hyperpigmentation, acrocyanosis, facial plethora, hemangiomas, hypertrichosis, and scleroderma-like skin changes.1,3 Glomeruloid hemangioma was first described by Chan et al. in 1990, and while it is uncommon, it constitutes the most specific skin manifestation of POEMS syndrome, with a strong association between both.4 It is believed to be a proliferative reaction to angiogenic stimuli (VEGF, interleukin 1, and tumor necrosis factor) and usually appears abruptly as multiple violaceous dome-shaped papules with a vascular appearance on the trunk and proximal area of the extremities. Sudden onset over a few days or weeks helps to establish an association between glomeruloid hemangioma and POEMS syndrome, since a slower onset may be observed in patients with no associated symptoms.3 Furthermore, it is important to stress that glomeruloid hemangiomas can precede full development of the disease by some years, thus providing an early sign of POEMS syndrome.5

Atypical, or incomplete, POEMS syndrome4,6 is characterized by the absence of one of the mandatory criteria. This condition requires close follow-up, since progression to the complete form has been reported, as in the case we present.

Treatment ranges from radiotherapy to systemic approaches similar to those used in multiple myeloma.

Conflicts of Interest

The authors declare that they have no conflicts of interest.

References
[1]
A. Dispenzieri.
POEMS syndrome: 2019 update on diagnosis, risk-stratification, and management.
Am J Hematol, 94 (2019), pp. 812-827
[2]
A. D'Souza, S.R. Hayman, F. Buadi, M. Mauermann, M.Q. Lacy, M.A. Gertz, et al.
The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome.
Blood, 118 (2011), pp. 4663-4665
[3]
I. Hernández Aragüés, A. Pulido Pérez, C. Ciudad Blanco, V. Parra Blanco, R. Suárez Fernández.
Hemangiomas glomeruloides y síndrome POEMS.
Actas Dermosifiliogr, 108 (2017), pp. e15-e19
[4]
Y. Charli-Joseph, M. Fernández-Sánchez, M. Saeb-Lima, R. Orozco-Topete.
POEMS syndrome: are current diagnostic criteria too exclusive?.
J Am Acad Dermatol, 65 (2011), pp. 415-417
[5]
J.Y. Lee, J.K. Choi, J.W. Ha, S.E. Park, C.W. Kim, S.S. Kim.
Glomeruloid hemangioma as a marker for the early diagnosis of POEMS syndrome.
Ann Dermatol, 29 (2017), pp. 249-251
[6]
T. He, A. Zhao, H. Zhao, H. Cai, J. Feng, L. Zhang, et al.
Clinical characteristics and the long-term outcome of patients with atypical POEMS syndrome variant with undetectable monoclonal gammopathy.
Ann Hematol, 98 (2019), pp. 735-743
Copyright © 2022. AEDV
Descargar PDF
Idiomas
Actas Dermo-Sifiliográficas
Opciones de artículo
Herramientas
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?