{"orpha":33577,"name":"Nodular non-suppurative panniculitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Idiopathic lobular panniculitis","Idiopathic nodular panniculitis","Pfeiffer-Weber-Christian syndrome","Relapsing febrile nodular nonsuppurative panniculitis","Relapsing febrile nodular panniculitis","WCD","Weber-Christian disease","Weber-Christian panniculitis"],"definition":"A rare skin disorder characterized by recurring inflammation in the subcutaneous layer of fat.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-33577/","orphanet_url":"https://www.orpha.net/en/disease/detail/33577","names":{"fr":{"name":"Panniculite nodulaire non suppurative","synonyms":["Maladie de Weber-Christian","Panniculite de Weber-Christian","Panniculite lobulaire idiopathique","Panniculite nodulaire fébrile récurrente","Panniculite nodulaire fébrile récurrente non suppurative","Panniculite nodulaire idiopathique","Syndrome de Pfeiffer-Weber-Christian"],"definition":"Maladie rare de la peau caractérisée par une inflammation récurrente du tissu adipeux sous-cutané."},"de":{"name":"Pannikulitis, noduläre nichteitrige","synonyms":["Noduläre nicht-suppurative Pannikulitis","Pannikulitis, febrile noduläre rekurrierende","Pannikulitis, idiopathische lobuläre","Pannikulitis, idiopathische noduläre","Pfeiffer-Weber-Christian-Syndrom","Weber-Christan-Krankheit","Weber-Christian Pannikulitis"],"definition":"Die Noduläre nicht-suppurative Pannikulitis, bekannt als Weber-Christan-Krankheit (WCD), ist eine seltene Hautkrankheit mit rezidivierenden Entzündungen des subkutanen Fettgewebes."},"es":{"name":"Paniculitis nodular no supurativa","synonyms":["Enfermedad de Weber-Christian (WCD)","Paniculitis febril nodular recurrente","Paniculitis febril nodular recurrente no supurativa","Paniculitis idiopática lobular","Paniculitis idiopática nodular","Síndrome de Pfeiffer-Weber-Christian"],"definition":"Es un trastorno cutáneo poco frecuente caracterizado por una inflamación recurrente en la capa de grasa subcutánea."},"it":{"name":"Panniculite nodulare non suppurativa","synonyms":["Malattia di Weber-Christian (WCD)","Panniculite di Weber-Christian","Panniculite lobulare idiopatica","Panniculite nodulare borreliosica","Panniculite nodulare borreliosica non suppurativa","Panniculite nodulare idiopatica","Sindrome di Pfeiffer-Weber-Christian"]},"nl":{"name":"Nodulaire niet-suppuratieve panniculitis","synonyms":["Idiopathische lobulaire panniculitis","Idiopathische nodulaire panniculitis","Panniculitis van Weber-Christian","Syndroom van Pfeiffer-Weber-Christian","Terugkerende febriele nodulaire niet-suppuratieve panniculitis","Terugkerende febriele nodulaire panniculitis","WCD","Ziekte van Weber-Christian"],"definition":"Een zeldzame aandoening van huid, gekenmerkt door recidiverende inflammatie in onderhuids vetweefsel."},"pt":{"name":"Paniculite não-supurativa nodular","synonyms":["Paniculite de Weber-Christian"]},"pl":{"name":"Nawracające nieropne zapalenie tkanki podskórnej","synonyms":["Choroba Webera i Christiana","Idiopatyczne guzkowe zapalenie tkanki podskórnej","Idiopatyczne płatowe zapalenie tkanki podskórnej","Nawracające gorączkowe guzkowe nieropne zapalenie tkanki podskórnej","Nawracające gorączkowe guzkowe zapalenie tkanki podskórnej","WCD","Zapalenie tkanki podskórnej Webera i Christiana","Zespół Pfeiffera, Webera i Christiana"]},"cs":{"name":"Nodulární nehnisavá panikulitida","synonyms":["Idiopatická lobulární panikulitida","Idiopatická nodulární panikulitida","Pfeifferova-Weberova-Christianova nemoc","Relabující febrilní nodulární nehnisavá panikulitida","Relabující febrilní nodulární panikulitida","WCD","Weberova-Christianova nemoc","Weberova-Christianova panikulitida","Pfeiffer-Weber-Christianova nemoc","Weber-Christianova nemoc","Weber-Christianova panikulitida"]},"uk":{"name":"Вузлуватий негнійний паннікуліт","synonyms":["Ідіопатичний лобулярний паннікуліт","Ідіопатичний вузликовий паннікуліт","Синдром Пфайфера-Вебера-Крістіана","Рецидивуючий гарячковий вузликовий ненагноительний паннікуліт","Рецидивуючий гарячковий вузликовий паннікуліт","WCD","Хвороба Вебера-Крістіана","Паннікуліт Вебера-Крістіана"],"definition":"A rare skin disorder characterized by recurring inflammation in the subcutaneous layer of fat."},"zh":{"name":"非化脓性结节性脂膜炎","synonyms":["复发性发热性结节性非化脓性脂膜炎"]},"tr":{"name":"Nodüler süpüratif olmayan pannikülit","synonyms":["WCD","Weber-Christian hastalığı","Weber-Christian pannikülit","İdiyopatik lobüler pannikülit","İdiyopatik nodüler pannikülit","Pfeiffer-Weber-Christian sendromu","Tekrarlayan ateşli nodüler pannikülit","Tekrarlayan ateşli nodüler, süpüratif olmayan pannikülit"]}},"xrefs":[{"source":"ICD-10","code":"M35.6","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"EF00.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"616271186"},{"source":"MONDO","code":"0018063","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D010201","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0030328","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7879","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10047883","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":["Not applicable"],"onset":["Adult"],"prevalence":[{"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:0002027","label":"Abdominal pain","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0008065","label":"Aplasia/Hypoplasia of the skin","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000969","label":"Edema","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:0003326","label":"Myalgia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012490","label":"Panniculitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001482","label":"Subcutaneous nodule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100533","label":"Inflammatory abnormality of the eye","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001744","label":"Splenomegaly","frequency":"Occasional (29-5%)","criteria":null}]}}