{"orpha":50918,"name":"Kikuchi-Fujimoto disease","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Histiocytic necrotizing lymphadenitis","Kikuchi disease"],"definition":"A rare systemic disease characterized by subacute, necrotizing, regional lymphadenopathy with tenderness, usually accompanied with mild fever, night sweats, myalgia, leukopenia and anemia. Less frequent symptoms include weight loss, nausea, vomiting and sore throat. It is a self-limiting benign disease that usually recedes within six months, even without drug treatment.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-50918/","orphanet_url":"https://www.orpha.net/en/disease/detail/50918","names":{"fr":{"name":"Maladie de Kikuchi-Fujimoto","synonyms":["Lymphadénite histiocytaire nécrosante","Maladie de Kikuchi"],"definition":"Maladie systémique rare caractérisée par une lymphadénopathie régionale subaiguë, nécrosante et sensible à la palpation, généralement accompagnée d'une fièvre légère, de sueurs nocturnes, de myalgie, de leucopénie et d'anémie. Les symptômes les moins fréquents sont la perte de poids, les nausées, les vomissements et les pharingites. Il s'agit d'une maladie bénigne, spontanément résolutive, qui disparaît généralement en 6 mois, même en l'absence de traitement médicamenteux."},"de":{"name":"Kikuchi-Fujimoto-Krankheit","synonyms":["Kikuchi-Fujimoto-Lymphadenitis","Lymphadenitis, histiozytäre nekrotisierende","Kikuchi-Krankheit"],"definition":"Eine seltene systemische Krankheit, die durch eine subakute, nekrotisierende, regionale Lymphadenopathie mit Druckempfindlichkeit gekennzeichnet ist und in der Regel mit leichtem Fieber, Nachtschweiß, Myalgie, Leukopenie und Anämie einhergeht. Zu den weniger häufigen Symptomen gehören Gewichtsverlust, Übelkeit, Erbrechen und Halsschmerzen. Es handelt sich um eine selbstlimitierende gutartige Erkrankung, die in der Regel innerhalb von sechs Monaten abklingt, auch ohne medikamentöse Behandlung."},"es":{"name":"Enfermedad de Kikuchi-Fujimoto","synonyms":["Enfermedad de Kikuchi"],"definition":"Es una enfermedad sistémica poco frecuente caracterizada por linfadenopatía necrosante regional subaguda, sensible a la palpación, generalmente acompañada de fiebre baja, sudores nocturnos, mialgia, leucopenia y anemia. Los síntomas menos frecuentes incluyen pérdida ponderal, náuseas, vómitos y faringitis. Es una enfermedad benigna autolimitada que suele remitir en un plazo de seis meses, incluso sin tratamiento farmacológico."},"it":{"name":"Malattia di Kikuchi-Fujimoto","synonyms":["Linfadenite istiocitica necrotizzante","Malattia di Kikuchi"]},"nl":{"name":"Ziekte van Kikuchi-Fujimoto","synonyms":["Hystiocytische necrotiserende lymfadenitis","Ziekte van Kikuchi"],"definition":"Een zeldzame systemische aandoening, gekenmerkt door subacute, necrotiserende, regionale lymfadenopathie met drukgevoeligheid, meestal vergezeld van milde koorts, nachtzweten, myalgie, leukopenie en anemie. Minder frequente symptomen zijn onder meer gewichtsverlies, misselijkheid, braken en pijnlijke keel. Het is een zelfbeperkende goedaardige ziekte die meestal verdwijnt binnen de zes maanden, zelf zonder behandeling met geneesmiddel."},"pt":{"name":"Doença Kikuchi-Fujimoto","synonyms":["Doença de Kikuchi","Linfadenite necrotizante histiocítica"],"definition":"Doença sistémica rara caracterizada por linfadenopatia regional subaguda e necrotizante, com sensibilidade, geralmente acompanhada de febre leve, suores noturnos, mialgias, leucopenia e anemia. Os sintomas menos frequentes incluem perda de peso, náuseas, vómitos e dor de garganta. Trata-se de uma doença benigna autolimitada que, normalmente, regride no prazo de seis meses, mesmo sem tratamento medicamentoso."},"pl":{"name":"Choroba Kikuchi i Fujimoto","synonyms":["Choroba Kikuchi","Histiocytarne martwicze zapalenie węzłów chłonnych"]},"cs":{"name":"Kikuchiho-Fujimotova choroba","synonyms":["Histiocytární nekrotizující lymfadenitida","Kikuchiho nemoc","Kikuchi-Fujimotova choroba"]},"uk":{"name":"Хвороба Кікучі-Фуджімото","synonyms":["Гістіоцитарний некротизуючий лімфаденіт","Хвороба Кікучі"],"definition":"A rare systemic disease characterized by subacute, necrotizing, regional lymphadenopathy with tenderness, usually accompanied with mild fever, night sweats, myalgia, leukopenia and anemia. Less frequent symptoms include weight loss, nausea, vomiting and sore throat. It is a self-limiting benign disease that usually recedes within six months, even without drug treatment."},"zh":{"name":"Kikuchi-Fujimoto病","synonyms":["组织细胞性坏死性淋巴结炎"]},"tr":{"name":"Kikuchi-Fujimoto hastalığı","synonyms":["Kikuchi hastalığı","Histiositik nekrotizan lenfadenit"]}},"xrefs":[{"source":"ICD-10","code":"I88.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"4B2Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2087666237"},{"source":"MONDO","code":"0018864","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D020042","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0398367","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6834","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10069070","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":[],"onset":["Adolescent","Adult","Childhood","Infancy"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_25500707[PMID]"},{"type":"Cases/families","qualification":"Case(s)","class":null,"value":1052.0,"area":"Worldwide","status":"Validated","source":"25500707[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.278,"area":"Martinique","status":"Validated","source":"27015112[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-08-04","source":"26627522[PMID]_27207311[PMID]","terms":[{"hpo":"HP:0025289","label":"Cervical lymphadenopathy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002733","label":"Abnormality of the lymph nodes","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0001596","label":"Alopecia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002039","label":"Anorexia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025143","label":"Chills","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000992","label":"Cutaneous photosensitivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025435","label":"Increased circulating lactate dehydrogenase concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001882","label":"Leukopenia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011134","label":"Low-grade fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0025300","label":"Malar rash","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030166","label":"Night sweats","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000155","label":"Oral ulcer","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100540","label":"Palpebral edema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200036","label":"Skin nodule","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002633","label":"Vasculitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200029","label":"Vasculitis in the skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008066","label":"Abnormal blistering of the skin","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006530","label":"Abnormal pulmonary interstitial morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011024","label":"Abnormality of the gastrointestinal tract","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000464","label":"Abnormality of the neck","frequency":"Occasional (29-5%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0001903","label":"Anemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003493","label":"Antinuclear antibody positivity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001875","label":"Decreased total neutrophil count","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011227","label":"Elevated circulating C-reactive protein concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002910","label":"Elevated circulating hepatic transaminase concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003565","label":"Elevated erythrocyte sedimentation rate","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011801","label":"Enlargement of parotid gland","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025475","label":"Erythematous macule","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100827","label":"Lymphocytosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012733","label":"Macule","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001287","label":"Meningitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200034","label":"Papule","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200041","label":"Skin erosion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200035","label":"Skin plaque","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001744","label":"Splenomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001251","label":"Ataxia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0008940","label":"Generalized lymphadenopathy","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012819","label":"Myocarditis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002202","label":"Pleural effusion","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0200039","label":"Pustule","frequency":"Very rare (<4-1%)","criteria":null}]}}