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Klippel-Trénaunay syndrome: Cutaneous manifestations
Síndrome de Klippel-Trénaunay: manifestaciones cutáneas
A. Rodríguez-Villa Lario
, A. Cabrera-Hernández, L. Trasobares-Marugán
Corresponding author
Servicio de Dermatología, Hospital Universitario Príncipe de Asturias, Universidad de Alcalá, Alcalá de Henares, Madrid, Spain
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"apellidos" => "Trasobares-Marugán" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Dermatología, Hospital Universitario Príncipe de Asturias, Universidad de Alcalá, Alcalá de Henares, Madrid, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome de Klippel-Trénaunay: manifestaciones cutáneas" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:6 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1068 "Ancho" => 1250 "Tamanyo" => 156855 ] ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 48-year-old man previously diagnosed with Klippel-Trénaunay syndrome but with no medical follow-up came to the emergency department complaining of abdominal pain that started hours ago related to an uncomplicated irreducible umbilical hernia.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The skin examination revealed numerous pink maculas in the upper dorsal region, with well-defined geographical edges that whitened when pressed. The examination also revealed a larger macula on the right flank exceeding the midline and a macula of similar characteristics on the lateral face of the right leg, with varicose veins in its interior. The patient also presented ipsilateral body hemihypertrophy (<a class="elsevierStyleCrossRefs" href="#fig0005">Figs. 1 and 2</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Klippel-Trénaunay syndrome is encompassed within the mixed or combined low-flow vascular malformations and is characterized by the combination of vascular malformations of capillary, lymphatic and venous strains, along with bone and soft tissue hypertrophy. The disease has been related to mutations of the PIK3CA gene, shows no predilection for a particular sex and has a sporadic onset, although familial cases have been reported.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Capillary malformations correspond in most cases with port wine stains predominantly on the lower extremities; involvement of the arms and chest is less common. Venous malformations include varicose veins, persistence of embryonic veins and valvular incompetence. Lymphedema can sometimes be observed with this condition. Clinicians need to be aware of the potential complications associated with this syndrome, such as bleeding of abnormal vessels in the gastrointestinal or genitourinary tract, as well as local and systemic thrombotic phenomena. Ulceration and infection of the affected limb can also appear.</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: Rodríguez-Villa Lario A, Cabrera-Hernández A, Trasobares-Marugán L. Síndrome de Klippel-Trénaunay: manifestaciones cutáneas. 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