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1</a>A&#41;&#46; Dermoscopy showed wine colored dots on a homogeneous pink background &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>B&#41;&#46;</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">Histology showed an orthokeratotic epidermis with acanthosis and papillomatosis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>A&#41; and dilated vessels in a lobular pattern&#44; surrounded by scant perivascular inflammatory infiltrate in the superficial and deep dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>B and C&#41;&#46; Immunohistochemical staining was positive for Wilms tumor 1 &#40;WT1&#41; and glucose transporter 1 &#40;GLUT1&#41; and negative for D2-40 &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Ultrasound showed that the lesions were superficial&#44; and revealed a slight increase in soft tissue thickness in the medial dorsal region of the right foot&#44; with no detectable flow&#46; A complete laboratory work-up revealed no alterations in the hemogram&#44; liver or kidney profile&#44; or D-dimer levels&#46;</p></span><span id="sec0021" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0021">What is Your Diagnosis&#63;</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Verrucous venous malformation &#40;VVM&#41;&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Daily topical treatment with 0&#46;5&#37; timolol was prescribed for 6 months&#44; but was discontinued in the absence of any improvement&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">VVM is a vascular anomaly&#46; There has been much discussion as to whether it constitutes a malformation or a tumor&#46; The lesions&#44; which are usually present at birth or appear in early childhood&#44; consist of single or multiple dark red patches&#46; These most frequently occur on the lower limbs&#44; tend to darken&#44; thicken&#44; and become hyperkeratotic&#44; and rarely undergo remission&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> VVM is characterized by compact hyperkeratosis of the epidermis and papillomatosis&#44; irregular acanthosis&#44; and dilated capillaries in the papillary and deep dermis and the subcutaneous cellular tissue&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Although its clinical characteristics resemble those of a vascular malformation&#44; it was initially considered a vascular tumor by the International Society for the Study of Vascular Anomalies due to its positive immunostaining for WT1 and GLUT1&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Clinically&#44; VVM can be confused with other vascular lesions&#44; such as angiokeratoma&#44; and with capillary&#44; venous&#44; and lymphatic vascular malformations that are negative for GLUT1 and WT1&#44; despite displaying hyperkeratosis and acanthosis&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Recently&#44; Couto et al&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> described the presence in VVM tissue of a somatic missense mutation in mitogen-activated protein kinase kinase kinase 3&#44; which is involved in the angiopoietin-1 and tunica intima endothelial cell kinase signaling cascade&#44; 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Case for Diagnosis
Violaceous erythematous plaques on a lower limb
Placas eritematovioláceas en miembro inferior
I. Villegas-Romeroa,
Corresponding author
imvr91@gmail.com

Corresponding author.
, K. Tello-Collantesb, D. Jiménez-Galloa
a Unidad de Gestión Clínica de Dermatología Médico-Quirúrgica y Venereología, Hospital Universitario Puerta del Mar, Cádiz, Spain
b Servicio de Anatomía Patológica, Hospital Universitario Puerta del Mar, Cádiz, Spain
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1</a>A&#41;&#46; Dermoscopy showed wine colored dots on a homogeneous pink background &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>B&#41;&#46;</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">Histology showed an orthokeratotic epidermis with acanthosis and papillomatosis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>A&#41; and dilated vessels in a lobular pattern&#44; surrounded by scant perivascular inflammatory infiltrate in the superficial and deep dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>B and C&#41;&#46; Immunohistochemical staining was positive for Wilms tumor 1 &#40;WT1&#41; and glucose transporter 1 &#40;GLUT1&#41; and negative for D2-40 &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Ultrasound showed that the lesions were superficial&#44; and revealed a slight increase in soft tissue thickness in the medial dorsal region of the right foot&#44; with no detectable flow&#46; A complete laboratory work-up revealed no alterations in the hemogram&#44; liver or kidney profile&#44; or D-dimer levels&#46;</p></span><span id="sec0021" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0021">What is Your Diagnosis&#63;</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Verrucous venous malformation &#40;VVM&#41;&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Daily topical treatment with 0&#46;5&#37; timolol was prescribed for 6 months&#44; but was discontinued in the absence of any improvement&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">VVM is a vascular anomaly&#46; There has been much discussion as to whether it constitutes a malformation or a tumor&#46; The lesions&#44; which are usually present at birth or appear in early childhood&#44; consist of single or multiple dark red patches&#46; These most frequently occur on the lower limbs&#44; tend to darken&#44; thicken&#44; and become hyperkeratotic&#44; and rarely undergo remission&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> VVM is characterized by compact hyperkeratosis of the epidermis and papillomatosis&#44; irregular acanthosis&#44; and dilated capillaries in the papillary and deep dermis and the subcutaneous cellular tissue&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Although its clinical characteristics resemble those of a vascular malformation&#44; it was initially considered a vascular tumor by the International Society for the Study of Vascular Anomalies due to its positive immunostaining for WT1 and GLUT1&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Clinically&#44; VVM can be confused with other vascular lesions&#44; such as angiokeratoma&#44; and with capillary&#44; venous&#44; and lymphatic vascular malformations that are negative for GLUT1 and WT1&#44; despite displaying hyperkeratosis and acanthosis&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Recently&#44; Couto et al&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> described the presence in VVM tissue of a somatic missense mutation in mitogen-activated protein kinase kinase kinase 3&#44; which is involved in the angiopoietin-1 and tunica intima endothelial cell kinase signaling cascade&#44; and is implicated in both hereditary and sporadic venous malformations&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Based on its clinical course and recent genetic findings&#44; VVM is currently considered a venous malformation&#44;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> despite its immunohistochemical characteristics&#46;</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of Interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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Article information
ISSN: 15782190
Original language: English
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Idiomas
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