{"orpha":889,"name":"Cutaneous small vessel vasculitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Cutaneous hypersensitivity vasculitis"],"definition":"A small vessel vasculitis characterized by neutrophilic inflammation predominantly limited to the superficial cutaneous postcapillary venules and without systemic vasculitis or glomerulonephritis. Typical presentation is of unifocal or multifocal palpable purpura on the lower extremities.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-889/","orphanet_url":"https://www.orpha.net/en/disease/detail/889","names":{"fr":{"name":"Vascularite des petits vaisseaux cutanés","synonyms":["Vascularite hypersensitive cutanée"],"definition":"Vascularite des petits vaisseaux caractérisée par une inflammation neutrophilique principalement limitée aux veinules postcapillaires cutanées superficielles, en l'absence de vascularite systémique ou glomérulonéphrite. Elle se traduit typiquement par un purpura palpable, isolé ou diffus, siégeant sur les membres inférieurs."},"de":{"name":"Kutane Vaskulitis kleiner Gefäße","synonyms":["Vaskulitis, kutane hypersensitive"],"definition":"Eine Vaskulitis der kleinen Gefäße, die durch eine neutrophile Entzündung gekennzeichnet ist, die vorwiegend auf die oberflächlichen kutanen postkapillären Venolen beschränkt ist und keine systemische Vaskulitis oder Glomerulonephritis aufweist. Typisch ist eine unifokale oder multifokale, tastbare Purpura an den unteren Extremitäten."},"es":{"name":"Vasculitis cutánea de pequeño vaso","synonyms":["Angeítis cutánea de pequeño vaso","Angeítis cutánea por hipersensibilidad","Vasculitis cutánea por hipersensibilidad"],"definition":"Es una vasculitis de pequeños vasos caracterizada por una inflamación neutrofílica limitada a las vénulas cutáneas postcapilares superficiales y sin vasculitis sistémica o glomerulonefritis. La presentación típica es una púrpura palpable uni o multifocal en las extremidades inferiores."},"it":{"name":"Vasculite cutanea dei piccoli vasi","synonyms":["Angioite ipersensibile","Vasculite cutanea dei piccoli vasi","Vasculite ipersensitiva cutanea","Vasculite leucocitoclastica cutanea"],"definition":"La vasculite cutanea dei piccoli vasi è caratterizzata da una infiammazione neutrofila limitata per lo più alle venule post-capillari superficiali della cute, in assenza di vasculite sistemica o glomerulonefrite. Di solito la malattia si manifesta con una porpora palpabile mono- o multifocale sugli arti inferiori."},"nl":{"name":"Cutane vasculitis van kleine bloedvaten","synonyms":["Cutane overgevoeligheidsvasculitis"],"definition":"Een vasculitis van kleine bloedvaten, gekarakteriseerd door neutrofiele inflammatie van hoofdzakelijk oppervlakkige cutane postcapillaire venulen en zonder systemische vasculitis of glomerulonefritis. De typische presentatie is unifocale of multifocale palpabele purpura op onderste ledematen."},"pt":{"name":"Vasculite dos pequenos vasos cutâneos","synonyms":["Vasculite cutânea"]},"pl":{"name":"Leukocytoklastyczne zapalenie naczyń skórnych","synonyms":["Skórne leukocystoklastyczne zapalenie naczyń","Zapalenie małych naczyń skórnych","Zapalenie naczyń skórnych z nadwrażliwością","Zapalenie naczyń z nadwrażliwością"]},"cs":{"name":"Kožní leukocytoklastická angiitida","synonyms":["Kožní hypersenzitivní vaskulitida","Kožní leukocytoklastická vaskulitida","Kožní vaskulitida malých cév","Hypersenzitivní angiitida"]},"uk":{"name":"Шкірний васкуліт дрібних судин","synonyms":["Шкірний васкуліт гіперчутливості"],"definition":"A small vessel vasculitis characterized by neutrophilic inflammation predominantly limited to the superficial cutaneous postcapillary venules and without systemic vasculitis or glomerulonephritis. Typical presentation is of unifocal or multifocal palpable purpura on the lower extremities."},"zh":{"name":"皮肤小血管炎","synonyms":["皮肤过敏性血管炎"]},"tr":{"name":"Kutanöz küçük damar vasküliti","synonyms":["Kutanöz aşırı duyarlılık vasküliti"]}},"xrefs":[{"source":"ICD-10","code":"M31.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EF40.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"71458216"},{"source":"MONDO","code":"0019509","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"19509","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0151436","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7851","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":156149,"name":"Immune complex mediated vasculitis","classifications":[196,198]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":2.7,"area":"Norway","status":"Validated","source":"9805179[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0000965","label":"Cutis marmorata","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100758","label":"Gangrene","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200034","label":"Papule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000979","label":"Purpura","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001581","label":"Recurrent skin infections","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001025","label":"Urticaria","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002633","label":"Vasculitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000163","label":"Abnormal oral cavity morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001482","label":"Subcutaneous nodule","frequency":"Occasional (29-5%)","criteria":null}]}}