{"orpha":94087,"name":"Cytophagic histiocytic panniculitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["CHP","Winkelmann cytophagic panniculitis"],"definition":"Cytophagic histiocytic panniculitis (CHP) is a very rare form of panniculitis manifesting as recurrent multiple subcutaneous nodules (which may progressively become ecchymotic and ulcerated), and histologically characterized by lobular panniculitis with lymphocytic and histiocytic infiltration in the subcutaneous adipose tissue.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-94087/","orphanet_url":"https://www.orpha.net/en/disease/detail/94087","names":{"fr":{"name":"Panniculite histiocytaire cytophagique","synonyms":["CHP","Panniculite cytophagique de Winkelmann"],"definition":"La panniculite histiocytaire cytophagique (PHC) est une forme très rare de panniculite se manifestant par de multiples nodules sous-cutanés récurrents (qui peuvent progressivement évoluer en ecchymoses ou en ulcères). Ils sont caractérisés au niveau histologique par une panniculite lobulaire avec infiltration lymphocytaire et histiocytaire du tissu adipeux sous-cutané."},"de":{"name":"Pannikulitis, histiozytäre zytophagische","synonyms":["CHP","Pannikulitis, zytophagische, Typ Winkelmann"],"definition":"Die Zytophagische histiozytäre Pannikulitis (CHP) ist eine seltene Form von Pannikulitis, die sich mit rezidivierenden multiplen subkutanen Knötchen manifestiert, die zunehmend Einblutungen (Ekchymosen) entwickeln und ulzerieren. Histologisch besteht im Unterhautfettgewebe eine lobuläre Pannikulitis mit lymphozytärer und histiozytärer Infiltration."},"es":{"name":"Paniculitis histiocítica citofágica","synonyms":["CHP","Paniculitis citofágica de Winkelmann"],"definition":"Es una forma muy poco frecuente de paniculitis que se manifiesta como nódulos subcutáneos múltiples recurrentes (que pueden convertirse progresivamente en equimóticos y ulcerados), e histológicamente caracterizada por paniculitis lobulillar con infiltrado linfocitario e histiocítico en el tejido adiposo subcutáneo."},"it":{"name":"Panniculite citofagica istiocitaria","synonyms":["CHP","Panniculite citofagica di Winkelmann"]},"nl":{"name":"Cytofagische histiocytische panniculitis","synonyms":["CHP","Cytofagische panniculitis van Winkelmann"],"definition":"Een zeer zeldzame vorm van panniculitis, zich manifesterend als recidiverende multipele subcutane noduli (die progressief ecchymose en ulceratie kunnen vertonen), en histologisch gekenmerkt door lobulaire panniculitis met lymfocytische en histiocytische infiltratie in subcutaan vetweefsel."},"pt":{"name":"Paniculite citofágica histiocítica"},"pl":{"name":"Cytophagic histiocytic panniculitis","synonyms":["CHP"]},"cs":{"name":"Cytofagická histiocytární panikulitida","synonyms":["CHP","Winkelmannova cytofagická panikulitida"]},"uk":{"name":"Цитофагічний гістіоцитарний панікуліт","synonyms":["CHP","Цитофагічний паннікуліт Вінкельмана"],"definition":"Cytophagic histiocytic panniculitis (CHP) is a very rare form of panniculitis manifesting as recurrent multiple subcutaneous nodules (which may progressively become ecchymotic and ulcerated), and histologically characterized by lobular panniculitis with lymphocytic and histiocytic infiltration in the subcutaneous adipose tissue."},"zh":{"name":"组织细胞吞噬性脂膜炎","synonyms":["Winkelmann吞噬性脂膜炎"],"definition":"细胞吞噬性组织细胞性脂膜炎（CHP）是一种非常罕见的脂膜炎，表现为复发性多发性皮下结节（可逐渐形成瘀斑和溃疡），组织学特征为小叶性脂膜炎，皮下脂肪组织有淋巴细胞和组织细胞浸润。"},"tr":{"name":"Sitofajik histiositik pannikülit","synonyms":["CHP","Winkelmann sitofajik pannikülit"]}},"xrefs":[{"source":"ICD-10","code":"M35.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EE8Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1978274002"},{"source":"MONDO","code":"0019789","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0406594","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10081137","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79382,"name":"Subcutaneous tissue disease","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Cases/families","qualification":"Case(s)","class":null,"value":100.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}