{"orpha":482,"name":"Kimura disease","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Eosinophilic lymphogranuloma"],"definition":"A rare systemic disease characterized by chronic inflammation of unknown etiology and occurring predominantly in males in the second to third decades of life, mainly in Southeast Asian countries. Patients present with painless soft tissue swelling most commonly involving the head and neck region, usually with salivary gland involvement (parotid and submandibular glands) and regional lymphadenopathy. Frequent laboratory findings are peripheral eosinophilia and raised serum IgE. The diagnosis is confirmed histopathologically by the presence of well-formed lymphoid follicles with eosinophilic infiltration and vascular proliferation in the biopsy.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-482/","orphanet_url":"https://www.orpha.net/en/disease/detail/482","names":{"fr":{"name":"Maladie de Kimura","synonyms":["Lymphogranulome éosinophile"],"definition":"Maladie systémique rare caractérisée par une inflammation chronique d'étiologie inconnue survenant principalement chez les hommes âgés de 20 à 30&nbsp;ans, originaires d'Asie du Sud-Est. Les patients présentent une tuméfaction indolore des tissus mouse le plus souvent dans la région de la tête et du cou, s'étendant généralement aux glandes salivaires (parotides et submandibulaires), et une lymphadénopathie localisée. Les analyses biochimiques sanguines mettent typiquement en évidence une éosinophilie périphérique et une augmentation du taux d'IgE sériques. Le diagnostic est conforté par une analyse histopathologique montrant la présence de follicules lymphoïdes bien formés, une infiltration éosinophile et une prolifération vasculaire visible à la biopsie."},"de":{"name":"Kimura-Krankheit","synonyms":["Lymphogranulom, eosinophiles"],"definition":"Eine seltene Systemerkrankung, die durch eine chronische Entzündung unbekannter Ätiologie gekennzeichnet ist und vor allem bei Männern im zweiten bis dritten Lebensjahrzehnt auftritt, hauptsächlich in südostasiatischen Ländern. Die Patienten stellen sich mit schmerzlosen Weichteilschwellungen vor, die meist die Kopf- und Halsregion betreffen, in der Regel mit Beteiligung der Speicheldrüsen (Ohrspeicheldrüse und submandibuläre Drüsen) und regionaler Lymphadenopathie. Häufige Laborbefunde sind periphere Eosinophilie und erhöhte Serum-IgE-Werte. Die Diagnose wird histopathologisch durch das Vorhandensein gut ausgebildeter lymphatischer Follikel mit eosinophiler Infiltration und Gefäßproliferation in der Biopsie bestätigt."},"es":{"name":"Enfermedad de Kimura","definition":"Es una enfermedad sistémica poco frecuente caracterizada por inflamación crónica de etiología desconocida y que ocurre predominantemente en varones en la segunda a tercera décadas de la vida, principalmente en países del sudeste asiático. Los pacientes presentan inflamación indolora de los tejidos blandos que afecta con mayor frecuencia a la región de la cabeza y del cuello, generalmente con afectación de las glándulas salivales (glándulas parótidas y submandibulares) y linfadenopatía regional. Los hallazgos de laboratorio más frecuentes son eosinofilia periférica y elevación de la IgE sérica. El diagnóstico se confirma histopatológicamente por la presencia de folículos linfoides bien formados con infiltración eosinófila y proliferación vascular en la biopsia."},"it":{"name":"Malattia di Kimura","synonyms":["Linfogranuloma eosinofilo"],"definition":"La malattia di Kimura è una malattia infiammatoria cronica benigna la cui eziologia non è nota. È presente soprattutto nei Paesi asiatici (molto raramente in quelli occidentali) e colpisce per lo più i maschi giovani. Di solito esordisce con una massa solitaria o con masse multiple sottocutanee non soffici, localizzate sulla testa o sul collo (in particolare nella zona preauricolare e sottomandibolare) e/o con linfoadenopatia generalizzata non dolorosa, spesso con interessamento delle ghiandole salivari. Di solito gli esami di laboratorio evidenziano eosinofilia ematica e livelli molto elevati di immunoglobuline E (IgE) nel siero. Spesso si associa a malattie autoinfiammatorie (colite ulcerosa, asma bronchiale) e ad una malattia renale."},"nl":{"name":"Ziekte van Kimura","synonyms":["Eosinofiel lymfogranuloom"],"definition":"Een zeldzame systemische aandoening, gekenmerkt door chronische inflammatie met ongekende etiologie en vooral optredend bij mannen in het tweede tot derde levensdecennium, vooral in Zuid-Aziatische landen. Patiënten presenteren zich met pijnloze zwelling van weke delen, vooral ter hoogte van hoofd en nek/hals, meestal met betrokkenheid van speekselklieren (parotisklier en onderkaakspeekselklier) en regionale lymfadenopathie. Frequente laboratoriumbevindingen zijn perifere eosinofilie en verhoogd IgE in serum. De diagnose wordt histopathologisch bevestigd door de aanwezigheid van goed gevormde lymfefollikels met eosinofiele infiltratie en vasculaire proliferatie in het biopt."},"pt":{"name":"Doença Kimura","synonyms":["Linfogranuloma eosinofílico"],"definition":"Doença sistémica rara caracterizada por inflamação crónica de etiologia desconhecida e ocorrendo predominantemente em homens na segunda a terceira décadas de vida, principalmente em países do Sudeste Asiático. Os doentes apresentam edema indolor de tecido mole, mais comummente envolvendo a região da cabeça e pescoço, geralmente com envolvimento de glândulas salivares (glândulas parótidas e submandibulares) e linfadenopatia regional. Os achados laboratoriais frequentes são eosinofilia periférica e IgE sérica elevada. O diagnóstico é confirmado histopatologicamente pela presença de folículos linfoides bem formados com infiltração eosinofílica e proliferação vascular na biópsia."},"pl":{"name":"Choroba Kimura","synonyms":["Eozynofilny ziarniniak limfoidalny"],"definition":"Rzadka choroba układowa charakteryzująca się przewlekłym stanem zapalnym o nieznanej etiologii, występująca głównie u mężczyzn w drugiej lub trzeciej dekadzie życia, głównie w krajach Azji Południowo-Wschodniej. Pacjenci zgłaszają się z bezbolesnym obrzękiem tkanek miękkich, najczęściej obejmującym obszar głowy i szyi, zwykle z zajęciem gruczołów ślinowych (ślinianek przyusznych i podżuchwowych) oraz regionalną limfadenopatią. W badaniach laboratoryjnych często obecna jest obwodowa eozynofilia i podwyższone stężenie IgE w surowicy. Rozpoznanie potwierdza się histopatologicznie, w biopsji widoczne są dobrze uformowane grudki chłonne z naciekiem eozynofilowym i proliferacją naczyniową."},"cs":{"name":"Kimurova choroba","synonyms":["Eozinofilní lymfogranulom"]},"uk":{"name":"Хвороба Кімура","synonyms":["Еозинофільна лімфогранулема"],"definition":"Kimura disease is a benign and chronic inflammatory disorder of unknown etiology, occurring mainly in Asian countries (very rarely in Western countries) and predominantly affecting young men, that usually presents with solitary or multiple non-tender subcutaneous masses in the head and neck region (in particular the preauricular and submandibular area) and/or generalized painless lymphadenopathy, often with salivary gland involvement. Characteristic laboratory findings include blood eosinophilia and markedly elevated serum immunoglobulin E (IgE) levels. It is often associated with autoinflammatory disorders (i.e. ulcerative colitis, bronchial asthma) and a co-existing renal disease."},"zh":{"name":"木村病","synonyms":["嗜酸性淋巴肉芽肿"]},"tr":{"name":"Kimura hastalığı","synonyms":["Eozinofilik lenfogranülom"]}},"xrefs":[{"source":"ICD-10","code":"D21.9","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"4B2Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1229046951"},{"source":"MONDO","code":"0018830","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D000082242","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0033838","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6835","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10048640","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":182222,"name":"Rare systemic disease","classifications":[196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":300.0,"area":"Worldwide","status":"Validated","source":"18808562[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-07-07","source":"15087670[PMID]","terms":[{"hpo":"HP:0002729","label":"Follicular hyperplasia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003212","label":"Increased circulating IgE concentration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001880","label":"Increased total eosinophil count","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010286","label":"Abnormal salivary gland morphology","frequency":"Occasional (29-5%)","criteria":null}]}}