37:
175:) in the pathogenesis of KFD. However, many independent studies have failed to identify the presence of these infectious agents in cases of Kikuchi lymphadenopathy. In addition, serologic tests including antibodies to a host of viruses have consistently proven noncontributory and no viral particles have been identified ultrastructurally. KFD is now proposed to be a nonspecific hyperimmune reaction to a variety of infectious, chemical, physical, and neoplastic agents. Other autoimmune conditions and manifestations such as
84:
120:. In sixty to ninety percent of cases, lymphadenopathy presents in the posterior cervical lymph nodes with diameter enlargement typically being between one and two centimeters, but up to seven centimeters has been reported in literature. Occasionally, the supraclavicular and axillary lymph nodes become swollen as well. Rarely,
302:
self-limiting nature. That a definite identification of KFD can only be done via a biopsy of affected tissues further suggests that cases go unrecognized or undiagnosed. It is mainly a disease of young adults (20–30 years), with a slight bias towards females. The cause of this disease is not known, although infectious and
96:
was described in 1972 in Japan. It is also known as histiocytic necrotizing lymphadenitis, Kikuchi necrotizing lymphadenitis, phagocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, and necrotizing lymphadenitis. Kikuchi disease occurs sporadically in people with no family history
301:
Kikuchi's disease is a very rare disease mainly seen in Japan. Isolated cases are reported in North
America, Europe, Asia, England, and at least two cases in New Zealand. It is possible that the prevalence of KFD is greater than is reported given lymphadenopathy can be overlooked and the disease's
297:
Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting disorder that typically affects the cervical lymph nodes. Recognition of this condition is crucial, especially because it can easily be mistaken for tuberculosis, lymphoma, or even adenocarcinoma. Awareness of this disorder helps prevent
306:
causes have been proposed. The course of the disease is generally benign and self-limiting. Lymph node enlargement usually resolves over several weeks to six months. The recurrence rate is about 3%. Death from
Kikuchi disease is extremely rare and usually occurs due to
852:
683:
Tanaka, T.; Ohmori, M.; Yasunaga, S.; Ohshima, K.; Kikuchi, M.; Sasazuki, T. (1999). "DNA typing of HLA class II genes (HLA-DR, -DQ and -DP) in
Japanese patients with histiocytic necrotizing lymphadentis (Kikuchi's disease) 8".
647:
Rosado, F. G.; Tang, Y. W.; Hasserjian, R. P.; McClain, C. M.; Wang, B; Mosse, C. A. (2013). "Kikuchi-Fujimoto lymphadenitis: Role of parvovirus B-19, Epstein-Barr virus, human herpesvirus 6, and human herpesvirus 8".
938:
289:
have been used. If the clinical course is more severe, with multiple flares of bulky enlarged cervical lymph nodes and fever, then a low-dose corticosteroid treatment has been suggested.
285:
Symptomatic measures aimed at relieving the distressing local and systemic complaints have been described as the main line of management of KFD. Analgesics, antipyretics, NSAIDs, and
128:
are seen. Often a bout of extreme fatigue can occur - often taking hold during latter parts of the day and the affected person can be more prone to fatigue from exercise.
931:
206:
class II genes appear more frequently in patients with
Kikuchi disease, suggesting that there may be a genetic predisposition to the proposed autoimmune response.
136:
Some studies have suggested a genetic predisposition to the proposed autoimmune response. Several infectious candidates have been associated with
Kikuchi disease.
1232:
382:
Kaushik V, Malik TH, Bishop PW, Jones PH (June 2004). "Histiocytic necrotising lymphadenitis (Kikuchi's disease): a rare cause of cervical lymphadenopathy".
924:
612:
Atwater AR, Longley BJ, Aughenbaugh WD (July 2008). "Kikuchi's disease: case report and systematic review of cutaneous and histopathologic presentations".
218:. Kikuchi disease is a self-limiting illness which has symptoms which may overlap with Hodgkin's lymphoma leading to misdiagnosis in some patients.
1019:
1091:
548:
509:
1162:
279:
282:(NSAIDs) are indicated for tender lymph nodes and fever, and corticosteroids are useful in severe extranodal or generalized disease.
1227:
355:
Rammohan A, Cherukuri SD, Manimaran AB, Manohar RR, Naidu RM (June 2012). "Kikuchi-Fujimoto
Disease: A Sheep in Wolf's Clothing".
1194:
36:
1107:
328:
184:
235:
1189:
1081:
223:
176:
864:
1222:
203:
1237:
1086:
904:
1147:
246:
The differential diagnosis of
Kikuchi disease includes systemic lupus erythematosus (SLE), disseminated
199:-mediated immune response in a genetically susceptible individual to a variety of nonspecific stimuli.
139:
Many theories exist about the cause of KFD. Microbial/viral or autoimmune causes have been suggested.
534:
Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes.
1184:
1174:
219:
100:
It was first described by Dr
Masahiro Kikuchi (1935–2012) in 1972 and independently by Y. Fujimoto.
83:
1167:
1046:
312:
556:
513:
1179:
1152:
1142:
1053:
969:
303:
152:
121:
916:
979:
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964:
947:
757:
701:
665:
629:
587:
491:
450:
399:
364:
231:
72:
195:
and cutaneous necrotizing vasculitis have been linked to KFD. KFD may represent an exuberant
1201:
1157:
1132:
1119:
1036:
1026:
996:
813:
747:
737:
693:
657:
621:
481:
440:
430:
391:
263:
1076:
113:
258:, and viral lymphadenitis. Clinical findings sometimes may include positive results for
986:
752:
725:
445:
418:
286:
164:
697:
395:
1216:
1058:
775:
316:
308:
1127:
1041:
893:
818:
801:
247:
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661:
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species have been implicated. More recently, growing evidence suggests a role for
64:
869:
1031:
579:
486:
469:
255:
52:
625:
1137:
1068:
227:
125:
60:
48:
44:
270:
antibodies. For other causes of lymph node enlargement, see lymphadenopathy.
888:
192:
77:
761:
742:
705:
669:
633:
591:
495:
454:
435:
403:
368:
1014:
899:
251:
117:
56:
844:
802:"An unusual cause of cervical lymphadenopathy: Kikuchi-Fujimoto disease"
1101:
991:
188:
1006:
956:
856:
215:
196:
172:
51:
with
Kikuchi disease showing the characteristic features (abundant
109:
160:
156:
920:
30:
Histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto disease
267:
259:
168:
470:"Enigmatic Kikuchi-Fujimoto disease: a comprehensive review"
278:
No specific cure is known. Treatment is largely supportive.
719:
717:
715:
234:
are usually negative, and may help in differentiation from
468:
Bosch X, Guilabert A, Miquel R, Campo E (July 2004).
834:
1118:
1067:
1005:
955:
879:
838:
553:
Genetic and Rare
Diseases Information Center (GARD)
71:
26:
21:
114:enlargement of the lymph nodes (lymphadenopathy)
806:Journal of Microbiology and Infectious Diseases
350:
348:
346:
344:
108:The signs and symptoms of Kikuchi disease are
932:
8:
800:Ulug, Mehmet; Aslan, Vahap (March 1, 2012).
939:
925:
917:
835:
298:misdiagnosis and inappropriate treatment.
124:and nervous system involvement resembling
82:
35:
18:
817:
751:
741:
485:
444:
434:
340:
214:It is diagnosed by lymph node excision
1020:Asplenia with cardiovascular anomalies
1233:Lymphoid-related cutaneous conditions
1092:Intranodal palisaded myofibroblastoma
7:
780:The Lecturio Medical Concept Library
724:Bosch X, Guilabert A (23 May 2006).
280:Nonsteroidal anti-inflammatory drugs
122:enlargement of the liver and spleen
14:
730:Orphanet Journal of Rare Diseases
698:10.1034/j.1399-0039.1999.540305.x
1195:Postmastectomy lymphangiosarcoma
1163:Lymphedema–distichiasis syndrome
1108:Template:Respiratory pathology
819:10.5799/ahinjs.02.2012.01.0036
329:Cutaneous lymphoid hyperplasia
1:
662:10.1016/j.humpath.2012.05.016
417:Bosch X, Guilabert A (2006).
396:10.1016/s1479-666x(04)80084-2
185:juvenile idiopathic arthritis
584:National Library of Medicine
357:J Otolaryngol Head Neck Surg
236:systemic lupus erythematosus
155:, as well as other viruses (
1190:Postinflammatory lymphedema
1082:Generalized lymphadenopathy
950:: organ and vessel diseases
487:10.1309/YF08-1L4T-KYWV-YVPQ
224:antiphospholipid antibodies
1254:
726:"Kikuchi-Fujimoto disease"
626:10.1016/j.jaad.2008.03.012
419:"Kikuchi-Fujimoto disease"
177:antiphospholipid syndrome
43:
34:
1228:Lymphatic organ diseases
578:Muhammad, Masab (2023).
204:Human leukocyte antigen
743:10.1186/1750-1172-1-18
436:10.1186/1750-1172-1-18
242:Differential diagnosis
220:Antinuclear antibodies
1148:Congenital lymphedema
614:J. Am. Acad. Dermatol
141:Mycobacterium szulgai
1185:Factitial lymphedema
1175:Secondary lymphedema
1087:Castleman's disease
474:Am. J. Clin. Pathol
423:Orphanet J Rare Dis
116:, skin rashes, and
1180:Bullous lymphedema
1153:Lymphedema praecox
1143:Primary lymphedema
1054:Splenic infarction
880:External resources
153:Epstein-Barr virus
104:Signs and symptoms
97:of the condition.
1210:
1209:
1120:Lymphatic vessels
980:DiGeorge syndrome
948:Lymphatic disease
914:
913:
776:"Lymphadenopathy"
580:"Kikuchi Disease"
549:"Kikuchi disease"
510:"kikuchi disease"
232:rheumatoid factor
91:
90:
16:Medical condition
1245:
1202:Waldmann disease
1168:Milroy's disease
1158:Lymphedema tarda
1133:Lymphangiectasia
1047:Banti's syndrome
1037:Wandering spleen
1027:Accessory spleen
997:Thymic carcinoma
941:
934:
927:
918:
836:
824:
823:
821:
797:
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772:
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680:
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673:
644:
638:
637:
609:
603:
602:
600:
598:
575:
569:
568:
566:
564:
555:. Archived from
545:
539:
536:Acta Hematol Jpn
532:
526:
525:
523:
521:
512:. Archived from
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499:
489:
465:
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352:
87:
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39:
19:
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1248:
1247:
1246:
1244:
1243:
1242:
1213:
1212:
1211:
1206:
1114:
1097:Kikuchi disease
1077:Lymphadenopathy
1063:
1001:
951:
945:
915:
910:
909:
875:
874:
847:
833:
828:
827:
799:
798:
794:
784:
782:
774:
773:
769:
723:
722:
713:
686:Tissue Antigens
682:
681:
677:
650:Human Pathology
646:
645:
641:
611:
610:
606:
596:
594:
577:
576:
572:
562:
560:
547:
546:
542:
538:1972;35:379–80.
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519:
517:
508:
507:
503:
467:
466:
462:
416:
415:
411:
381:
380:
376:
354:
353:
342:
337:
325:
295:
287:corticosteroids
276:
244:
212:
134:
132:Pathophysiology
106:
94:Kikuchi disease
81:
22:Kikuchi disease
17:
12:
11:
5:
1251:
1249:
1241:
1240:
1235:
1230:
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1223:Histopathology
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1214:
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1207:
1205:
1204:
1199:
1198:
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1172:
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1155:
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1116:
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1111:
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1099:
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1084:
1079:
1073:
1071:
1065:
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1062:
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1056:
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1050:
1049:
1039:
1034:
1029:
1024:
1023:
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1009:
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1002:
1000:
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994:
989:
987:Ectopic thymus
984:
983:
982:
972:
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961:
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952:
946:
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943:
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861:
848:
843:
842:
840:
839:Classification
832:
831:External links
829:
826:
825:
792:
767:
711:
692:(3): 246–253.
675:
639:
604:
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333:
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211:
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165:parvovirus B19
133:
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105:
102:
89:
88:
75:
69:
68:
41:
40:
32:
31:
28:
24:
23:
15:
13:
10:
9:
6:
4:
3:
2:
1250:
1239:
1238:Rare diseases
1236:
1234:
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1203:
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1088:
1085:
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1080:
1078:
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1059:Splenic tumor
1057:
1055:
1052:
1048:
1045:
1044:
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1038:
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1021:
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1016:
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949:
942:
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837:
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735:
731:
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718:
716:
712:
707:
703:
699:
695:
691:
687:
679:
676:
671:
667:
663:
659:
656:(2): 255–59.
655:
651:
643:
640:
635:
631:
627:
623:
620:(1): 130–36.
619:
615:
608:
605:
593:
589:
585:
581:
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571:
559:on 8 May 2019
558:
554:
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544:
541:
537:
531:
528:
516:on 8 May 2019
515:
511:
505:
502:
497:
493:
488:
483:
480:(1): 141–52.
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452:
447:
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437:
432:
428:
424:
420:
413:
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405:
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390:(3): 179–82.
389:
385:
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375:
370:
366:
363:(3): 222–26.
362:
358:
351:
349:
347:
345:
341:
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330:
327:
326:
322:
320:
318:
317:heart failure
314:
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273:
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66:
65:H&E stain
62:
58:
54:
50:
46:
42:
38:
33:
29:
25:
20:
1128:Lymphangitis
1096:
1042:Splenomegaly
898:
887:
863:
851:
812:(1): 21–25.
809:
805:
795:
783:. Retrieved
779:
770:
733:
729:
689:
685:
678:
653:
649:
642:
617:
613:
607:
595:. Retrieved
583:
573:
561:. Retrieved
557:the original
552:
543:
535:
530:
518:. Retrieved
514:the original
504:
477:
473:
463:
426:
422:
412:
387:
383:
377:
360:
356:
300:
296:
293:Epidemiology
284:
277:
248:tuberculosis
245:
213:
201:
187:, bilateral
181:polymyositis
148:
144:
140:
138:
135:
107:
99:
93:
92:
1032:Polysplenia
970:Hyperplasia
597:17 November
313:respiratory
256:sarcoidosis
183:, systemic
61:neutrophils
53:histiocytes
27:Other names
1217:Categories
1138:Lymphedema
1069:Lymph node
975:Hypoplasia
736:(18): 18.
335:References
304:autoimmune
274:Management
228:anti-dsDNA
149:Toxoplasma
126:meningitis
49:lymph node
45:Micrograph
889:eMedicine
210:Diagnosis
193:arthritis
78:Angiology
73:Specialty
1015:Asplenia
900:Orphanet
894:med/3663
785:7 August
762:16722618
706:10519361
670:22939574
634:18462833
592:28613580
563:17 April
520:17 April
496:15272543
455:16722618
404:15570824
369:22762705
323:See also
252:lymphoma
202:Certain
145:Yersinia
118:headache
59:without
57:necrosis
1102:Tonsils
992:Thymoma
965:Abscess
870:D020042
860:: I88.1
753:1481509
446:1481509
384:Surgeon
189:uveitis
1007:Spleen
957:Thymus
760:
750:
704:
668:
632:
590:
494:
453:
443:
429:: 18.
402:
367:
230:, and
216:biopsy
197:T-cell
173:HTLV-1
80:
905:50918
315:, or
309:liver
110:fever
47:of a
1106:see
865:MeSH
787:2021
758:PMID
702:PMID
666:PMID
630:PMID
599:2022
588:PMID
565:2018
522:2018
492:PMID
451:PMID
400:PMID
365:PMID
171:and
161:HHV8
157:HHV6
147:and
143:and
853:ICD
814:doi
748:PMC
738:doi
694:doi
658:doi
622:doi
482:doi
478:122
441:PMC
431:doi
392:doi
268:IgA
264:IgG
260:IgM
169:HIV
63:).
1219::
903::
892::
868::
857:10
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804:.
778:.
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728:.
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618:59
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439:.
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427:1
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266:/
262:/
67:.
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