{"orpha":464318,"name":"Verrucous venous malformation","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Verrucous haemangioma","Verrucous hemangioma"],"definition":"A rare vascular anomaly characterized by congenital, solitary or grouped, red-to-purple plaques which may bleed and enlarge over time. The lesions show a predilection for the lower extremities. Histological examination reveals numerous dilated, congested capillaries and venules in the papillary dermis, often with a deep dermal component, and with increased density of variably congested capillaries and venules also in the subcutaneous tissue. The overlying epidermis displays prominent acanthosis, papillomatosis, hyperkeratosis, parakeratosis, and crusting.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-464318/","orphanet_url":"https://www.orpha.net/en/disease/detail/464318","names":{"fr":{"name":"Malformation veineuse verruqueuse","synonyms":["Hémangiome verruqueux"],"definition":"Anomalie vasculaire rare caractérisée par des plaques congénitales, solitaires ou groupées, de couleur rougeâtre à violacée, pouvant saigner et s'étendre avec le temps. Les sites de prédilection des lésions sont les membres inférieurs. L'examen histologique révèle une dilatation et une congestion de nombreux capillaires et veinules du derme papillaire et, souvent, une composante dermique profonde, ainsi qu'une congestion variable et une grande densité des capillaires et des veinules dans le tissu sous-cutané. L'épiderme sus-jacent présente une importante acanthose, une papillomatose, une hyperkératose, une parakératose et des croûtes."},"de":{"name":"Verruköse venöse Fehlbildung","synonyms":["Venöse Malformation, verruköse"],"definition":"Eine seltene Gefäßanomalie, die durch angeborene, einzelne oder gruppierte, rote bis violette Plaques gekennzeichnet ist, die bluten und sich mit der Zeit vergrößern können. Die Läsionen treten bevorzugt an den unteren Extremitäten auf. Die histologische Untersuchung zeigt zahlreiche erweiterte, verstopfte Kapillaren und Venolen in der papillären Dermis, oft mit einer tiefen dermalen Komponente, und mit erhöhter Dichte von unterschiedlich verstopften Kapillaren und Venolen auch im subkutanen Gewebe. Die darüber liegende Epidermis weist eine ausgeprägte Akanthose, Papillomatose, Hyperkeratose, Parakeratose und Krustenbildung auf."},"es":{"name":"Malformación venosa verrugosa","synonyms":["Hemangioma verrrugoso"],"definition":"Es una anomalía vascular poco frecuente caracterizada por placas congénitas, aisladas o agrupadas, de color rojo a púrpura que pueden sangrar y aumentar de tamaño con el tiempo. Las lesiones muestran predilección por las extremidades inferiores. El examen histológico revela numerosos capilares y vénulas dilatados y congestionados en la dermis papilar, a menudo con un componente dérmico profundo, y con un aumento de la densidad de capilares y vénulas de congestión variable también en el tejido subcutáneo. La epidermis suprayacente muestra una acantosis prominente, papilomatosis, hiperqueratosis, paraqueratosis y costras."},"it":{"name":"Malformazione verrucosa venosa","synonyms":["Emangioma verrucoso"],"definition":"Si tratta di un'anomalia vascolare rara, caratterizzata da placche congenite, solitarie o raggruppate, di colore rosso-violaceo, che possono sanguinare e allargarsi con il tempo. Le lesioni si localizzano prevalentemente sugli arti inferiori. L'esame istologico evidenzia numerosi capillari e venule dilatate e congestionate nel derma papillare, spesso con una componente dermica profonda, e una maggiore densità di capillari e venule variabilmente congestionate, anche nel tessuto sottocutaneo. L'epidermide sovrastante presenta una marcata acantosi, papillomatosi, ipercheratosi, paracheratosi e croste."},"nl":{"name":"Verruceuze veneuze malformatie","synonyms":["Verruceus hemangioom"],"definition":"Een zeldzame vasculaire anomalie, gekarakteriseerd door congenitale, solitaire of gegroepeerde, rode tot paarse plaques die mogelijk bloeden en mettertijd groter worden. De laesies komen eerder voor op onderste ledematen. Histologisch onderzoek toont talrijke gedilateerde, verstopte capillairen en venulen in papillare dermis, vaak met een diepe dermale component, en ook verhoogde densiteit van variabel verstopte capillairen en venulen in onderhuids weefsel. Bovenliggende epidermis vertoont prominente acanthose, papillomatose, hyperkeratose, parakeratose, en korstvorming."},"pt":{"name":"Hemangioma verrugoso"},"pl":{"name":"Naczyniak brodawkowaty","synonyms":["Verrucous haemangioma","Verrucous hemangioma"]},"cs":{"name":"Verukózní hemangiom","synonyms":["Verrucous haemangioma","Verrucous hemangioma"]},"uk":{"name":"Веррукозна гемангіома","definition":"A rare vascular anomaly characterized by congenital, solitary or grouped, red-to-purple plaques which may bleed and enlarge over time. The lesions show a predilection for the lower extremities. Histological examination reveals numerous dilated, congested capillaries and venules in the papillary dermis, often with a deep dermal component, and with increased density of variably congested capillaries and venules also in the subcutaneous tissue. The overlying epidermis displays prominent acanthosis, papillomatosis, hyperkeratosis, parakeratosis, and crusting."},"zh":{"name":"疣状血管瘤"},"tr":{"name":"Verrüköz hemanjiyom"}},"xrefs":[{"source":"ICD-10","code":"D18.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LC52","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1679975821"},{"source":"MONDO","code":"0018734","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0334540","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98028,"name":"Rare circulatory system disease"},"parents":[{"orpha":723518,"name":"Isolated combined vascular malformation","classifications":[147,198]},{"orpha":715334,"name":"Unifocal peripheral venous malformation","classifications":[147,198]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_25263605[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-04-29","source":"23578182[PMID]_21649690[PMID]","terms":[{"hpo":"HP:0001028","label":"Hemangioma","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0025092","label":"Epidermal acanthosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0045059","label":"Hyperkeratotic papule","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011123","label":"Inflammatory abnormality of the skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012740","label":"Papilloma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011356","label":"Regional abnormality of skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200035","label":"Skin plaque","frequency":"Frequent (79-30%)","criteria":null}]}}