{"orpha":49566,"name":"Acquired purpura fulminans","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A life-threatening, rapidly progressive thrombotic disorder affecting mainly neonates and children that is characterized by purpuric skin lesions and disseminated intravascular coagulation. It may progress rapidly to multi-organ failure caused by thrombotic occlusion of small and medium-sized blood vessels. There are two forms of the disorder that are classified according to triggering mechanisms: acute infectious (the most common form), and idiopathic purpura fulminans.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-49566/","orphanet_url":"https://www.orpha.net/en/disease/detail/49566","names":{"fr":{"name":"Purpura fulminans acquis","definition":"Maladie thrombotique rare, rapidement progressive et potentiellement létale, affectant principalement les nouveau-nés et les enfants, qui se caractérise par des lésions cutanées purpuriques et une coagulation intravasculaire disséminée. Elle peut évoluer rapidement vers une défaillance de plusieurs organes causée par l'occlusion thrombotique de vaisseaux sanguins de petit et moyen calibre. Il existe deux formes de la maladie classées en fonction des mécanismes déclencheurs : le purpura aigu infectieux (la forme la plus courante) et le purpura fulminans idiopathique."},"de":{"name":"Purpura fulminans, erworbene","definition":"Eine lebensbedrohliche, schnell fortschreitende thrombotische Erkrankung, die hauptsächlich Neugeborene und Kinder betrifft und durch purpurische Hautläsionen und disseminierte intravasale Gerinnung gekennzeichnet ist. Sie kann schnell zu einem Multiorganversagen führen, das durch einen thrombotischen Verschluss kleiner und mittelgroßer Blutgefäße verursacht wird. Es gibt zwei Formen der Erkrankung, die nach den auslösenden Mechanismen klassifiziert werden: akute infektiöse Purpura (die häufigste Form) und idiopathische Purpura fulminans."},"es":{"name":"Púrpura fulminante adquirido","definition":"Es un trastorno trombótico rápidamente progresivo y potencialmente mortal que afecta principalmente a recién nacidos y niños y que se caracteriza por lesiones cutáneas purpúricas y coagulación intravascular diseminada. Puede progresar rápidamente a fallo multiorgánico causada por oclusión trombótica de vasos sanguíneos de pequeño y mediano calibre. Hay dos formas del trastorno que se clasifican según los mecanismos desencadenantes: infecciosa aguda (la forma más común) y púrpura fulminante idiopática."},"it":{"name":"Porpora fulminante acquisita"},"nl":{"name":"Verworven purpura fulminans","definition":"Een levensbedreigende, snel progressieve trombotische aandoening die vooral neonaten en kinderen treft, gekenmerkt door purpurische huidlaesies en gedissemineerde intravasculaire coagulatie. Mogelijk is er snelle progressie naar multi-orgaanfalen, veroorzaakt door trombotische occlusie van kleine en middelgrote bloedvaten. Er zijn twee vormen van de aandoening, die geclassificeerd worden volgens uitlokkend mechanisme: acute infectieuze (de meest gangbare vorm) en idiopathische purpura fulminans."},"pt":{"name":"Púrpura fulminante"},"pl":{"name":"plamica piorunująca nabyta","definition":"Zagrażająca życiu, szybko postępująca choroba zakrzepowa, która dotyczy głównie noworodków i dzieci i charakteryzuje się zmianami skórnymi o typie plamicy oraz rozsianym wykrzepianiem wewnątrznaczyniowym. Może szybko przejść w niewydolność wielonarządową, spowodowaną zakrzepowym zamknięciem małych i średnich naczyń krwionośnych. Istnieją dwie postaci schorzenia, które sklasyfikowano według mechanizmów wyzwalających : ostra infekcyjna (najczęstsza postać) oraz idiopatyczna plamica piorunująca."},"cs":{"name":"Získaná purpura fulminans"},"uk":{"name":"Набута фульминантна пурпура","definition":"A life-threatening, rapidly progressive thrombotic disorder affecting mainly neonates and children that is characterized by purpuric skin lesions and disseminated intravascular coagulation. It may progress rapidly to multi-organ failure caused by thrombotic occlusion of small and medium-sized blood vessels. There are two forms of the disorder that are classified according to triggering mechanisms: acute infectious (the most common form), and idiopathic purpura fulminans."},"zh":{"name":"获得性暴发性紫癜"},"tr":{"name":"Edinsel purpura fulminans"}},"xrefs":[{"source":"ICD-10","code":"D65","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"3B20","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018854","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4510896","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10037556","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97992,"name":"Rare hematologic disease"},"parents":[{"orpha":248365,"name":"Rare thrombotic disorder due to an acquired coagulation factors defect","classifications":[194]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Childhood","Infancy","Neonatal"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-05-22","source":"2142890[PMID]_17679737[PMID]_20034317[PMID]_23582572[PMID]_29157918[PMID]","terms":[{"hpo":"HP:0008066","label":"Abnormal blistering of the skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001977","label":"Abnormal thrombosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001063","label":"Acrocyanosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025022","label":"Decreased erythrocyte sedimentation rate","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005521","label":"Disseminated intravascular coagulation","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011227","label":"Elevated circulating C-reactive protein concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025475","label":"Erythematous macule","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100758","label":"Gangrene","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011900","label":"Hypofibrinogenemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002958","label":"Immune dysregulation","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031365","label":"Macular purpura","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012733","label":"Macule","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003645","label":"Prolonged partial thromboplastin time","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008151","label":"Prolonged prothrombin time","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005543","label":"Reduced protein C activity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004855","label":"Reduced protein S activity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031273","label":"Shock","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001399","label":"Hepatic failure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011029","label":"Internal hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002170","label":"Intracranial hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002664","label":"Neoplasm","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100806","label":"Sepsis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025452","label":"Pyoderma gangrenosum","frequency":"Very rare (<4-1%)","criteria":null}]}}