Kikuchi disease
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Histiocytic necrotizing lymphadenitis; Kikuchi-Fujimoto disease; Kikuchi necrotizing lymphadenitis; phagocytic necrotizing lymphadenitis; subacute necrotizing lymphadenitis; necrotizing lymphadenitis
Overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Kikuchi’s disease (histiocytic necrotizing lymphadenitis) is a rare, non-cancerous enlargement of the lymph nodes: the diagnosis can be confirmed by lymph node biopsy.
Historical Perspective
Kikuchi-Fujimoto disease (KFD) was described in 1972 in Japan by Dr Masahiro Kikuchi in 1972[1] and independently by Y. Fujimoto.
Causes
The cause for Kikuchi disease has not been established although infectious and autoimmune etiologies have been proposed.
Diagnosis
Laboratory Findings
Kikuchi disease is diagnosed by lymph node excisional biopsy. ANA, APLA, anti-dsDNA, RF are usually negative, and may help in differentiating from SLE. Laboratory findings may sometimes include positive results for IgM, IgG or IgA antibodies.
Treatment
Medical Therapy
There is no specific cure for Kikuchi disease. Treatment is largely supportive. NSAIDs are useful if patient presents with tender lymph nodes and fever. Symptomatic measures aimed at relieving the distressing local and systemic complaints have been described as the main line of management of Kikuchi disease. Analgesics, antipyretics, nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids have been used. Corticosteroids are useful in severe extranodal or generalized disease. Multiple flares of bulky cervical lymphadenopathy and fever are treated with a low-dose corticosteroid.
References
- ↑ Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes. Acta Hematol Jpn 1972;35:379–80.
Historical Perspective
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Kikuchi-Fujimoto disease (KFD) was described in 1972 in Japan by Dr Masahiro Kikuchi in 1972[1] and independently by Y. Fujimoto.
References
- ↑ Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes. Acta Hematol Jpn 1972;35:379–80.
Pathophysiology
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Pathophysiology
Some studies have suggested a genetic predisposition to the proposed autoimmune response. Several infectious candidates have been associated with Kikuchi’s disease[1].
There are many postulates about the etiology of Kikuchi disease. A microbial/ viral or autoimmune cause has been suggested. Mycobacterium szulgai, Yersinia and Toxoplasma have been implicated. More recently there has been growing evidence of the role of Epstein-Barr virus, as well as other viruses (HHV6, HHV8, Parvovirus B19, HIV– and HTLV-1) in the pathogenesis of Kikuchi disease. [2] But serologic tests including antibodies to a host of viruses have consistently proven noncontributory and no viral particles have been identified ultrastructurally.
It is now proposed that Kikuchi disease is a nonspecific hyperimmune reaction to a variety of infectious, chemical, physical and neoplastic agents. It is possible that Kikuchi disease may represent an exuberant T-cell mediated immune response in a genetically susceptible individual to a variety of non-specific stimuli. [2]
Associated Conditions
Kikuchi’s disease has been reported to be associated with infections such as:
In addition to SLE, other autoimmune conditions and manifestations such as antiphospholipid syndrome, polymyositis, systemic juvenile idiopathic arthritis, bilateral uveitis, arthritis and cutaneous necrotizing vasculitis have been linked to Kikuchi disease.
References
- ↑ Atwater AR, Longley BJ, Aughenbaugh WD (2008). “Kikuchi’s disease: case report and systematic review of cutaneous and histopathologic presentations”. J Am Acad Dermatol. 59 (1): 130–6. doi:10.1016/j.jaad.2008.03.012. PMID 18462833.
- ↑ 2.0 2.1 Rammohan A, Cherukuri SD, Manimaran AB, Manohar RR, Naidu RM (2012). “Kikuchi-Fujimoto Disease: A Sheep in Wolf’s Clothing”. J Otolaryngol Head Neck Surg. 41 (3): 222–226. PMID 22762705. Unknown parameter
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Causes
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
The cause for Kikuchi disease has not been established although infectious and autoimmune etiologies have been proposed.
References
Differentiating Kikuchi disease from other Diseases
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Mahshid Mir, M.D. [2]
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Differentiating Kikuchi disease from other Diseases
Kikuchi disease 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:
Awareness of this disorder will help prevent misdiagnosis and inappropriate treatment.[1]
Abbreviations: ANA: Antinuclear antibody, RF: Rheumatoid factor, Anti-CCp: Anti-cyclic citrullinated protein antibody, Anti U1RNP: Anti-U1 ribonucleoprotein antibodies , Anti Sm : Anti-Sm antibodies, Anti Ro: Anti Ro antibody also called anti-Sjögren’s-syndrome-related antigen A antibody, Anti-dsDNA: Anti-double stranded DNA.
| Disease | Arthritis | Auto-antibodies | Raynaud phenomenon | Rash pattern | Distinguishing/specific features | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Polyarthritis | Tenderness | Edema | Deformity /Erosion | Pattern | ANA | RF | Anti-CCp | Anti U1RNP | Anti Sm | Anti Ro | Anti-dsDNA | |||||
| Kikuchi’s disease[2] | – | +/- | – | – | medium and large joints | ↑/↓ | – | – | – | – | – | – | – | Transient skin rashes, malar rash, erythematous macules, patches, papules, or plaques | May be associated with SLE | |
| Systemic lupus erythematosus[3] | + | + | + | – | Small joints | ↑ | – | – | – | ↑ | ↑ | – | + | Malar rash and photosensitivity | ||
| Rheumatoid arthritis (RA)[4] | + | + | + | + | Small and large joints | – | ↑↑ | ↑↑ | – | – | – | – | + | Subcutaneous nodules | Erosive arthropathy | |
| Rhupus[5] | + | + | + | + | Small and large joints | ↑ | ↑ | ↑ | ↑ | ↑ | – | ↑ | + | Malar rash and photosensitivity | Erosive arthropathy | |
| Mixed connective tissue disease (MCTD)[6] | – | – | – | + | Small and large joints | – | ↑↑ | ↑ | – | – | – | – | + | Cutaneous eruptions, gottron’s papules, photodistributed erythema, poikiloderma, and calcinosis cutis | Overlapping features of SLE, systemic sclerosis (SSc), and polymyositis (PM) that lead to more than one diagnosis | |
| Undifferentiated connective tissue disease (UCTD)[7] | + | – | – | – | Lower extremity | ↑ | ↑ | – | – | ↑ | – | – | + | Erythematous macules, patches, or papules with delicate scale | Multiple connective tissue diseases with no enough criteria for a single diagnosis | |
| Systemic sclerosis (SSc)[8] | +/- | + | + | +/- | Lower extremity | ↑↑ | – | – | – | ↑ | – | ↑ | + | Hyperkeratosis, edema, and erythema | Sclerodactyly, Telangiectasias, Calcinosis, Malignant hypertension, acute renal failure | |
| Sjögren’s syndrome[9] | +/- | +/- | – | – | Lower extremity, axiallary creases | ↑ | – | – | – | ↑ | ↑ | – | – | Xerosis, scaly skin, annular erythema | Keratoconjunctivitis sicca | |
| Vasculitis | Giant cell[10] | – | + | + | – | Distal extremity | – | – | – | – | – | – | – | – | Rare | Involvement of cranial branches of arteries, visual loss |
| Takayasu[11] | – | +/- | +/- | – | Transient extremity | – | – | – | – | – | – | – | Erythema nodosum, pyoderma gangrenosum | Absent or weak peripheral pulse | ||
| Poly-arteritis nodosa[12] | – | +/- | – | – | General and mild | – | – | – | – | – | – | – | Tender erythematous nodules, purpura, livedo reticularis, bullous or vesicular eruption | Testicular pain or tenderness and neuropathies | ||
| Behçet’s syndrome[13] | +/- | +/- | +/- | – | medium and large joints | – | – | – | – | – | – | – | – | Recurrent and usually painful mucocutaneous ulcers, acneiform lesions, papulo-vesiculo-pustular eruptions, superficial thrombophlebitis | Male dominancy | |
| Serum sickness[14] | + | + | +/- | – | General | – | – | – | – | – | – | – | – | Pruritic rash, urticaria and/or serpiginous macular rash | Self-limited | |
| Psoriatic arthritis[15] | – | – | – | – | Small and large joints | – | – | – | – | – | – | – | – | Psoriasis and onychodystrophy | Dactylitis (sausage digits) | |
| Human parvovirus B19 infection[16] | + | + | – | – | Small joints | – | – | – | – | – | – | – | – | Erythematous rashes | Rare in adults, fifth’s disease in children | |
References
- ↑ Rammohan A, Cherukuri SD, Manimaran AB, Manohar RR, Naidu RM (2012). “Kikuchi-Fujimoto Disease: A Sheep in Wolf’s Clothing”. J Otolaryngol Head Neck Surg. 41 (3): 222–226. PMID 22762705. Unknown parameter
|month=ignored (help) - ↑ Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M (2007). “Kikuchi-Fujimoto Disease: analysis of 244 cases”. Clin. Rheumatol. 26 (1): 50–4. doi:10.1007/s10067-006-0230-5. PMID 16538388.
- ↑ Ehmke TA, Cherian JJ, Wu ES, Jauregui JJ, Banerjee S, Mont MA (2014). “Treatment of osteonecrosis in systemic lupus erythematosus: a review”. Curr Rheumatol Rep. 16 (9): 441. doi:10.1007/s11926-014-0441-8. PMID 25074031.
- ↑ Lee DM, Weinblatt ME (2001). “Rheumatoid arthritis”. Lancet. 358 (9285): 903–11. doi:10.1016/S0140-6736(01)06075-5. PMID 11567728.
- ↑ Panush RS, Edwards NL, Longley S, Webster E (1988). “‘Rhupus’ syndrome”. Arch. Intern. Med. 148 (7): 1633–6. PMID 3382309.
- ↑ Cappelli S, Bellando Randone S, Martinović D, Tamas MM, Pasalić K, Allanore Y, Mosca M, Talarico R, Opris D, Kiss CG, Tausche AK, Cardarelli S, Riccieri V, Koneva O, Cuomo G, Becker MO, Sulli A, Guiducci S, Radić M, Bombardieri S, Aringer M, Cozzi F, Valesini G, Ananyeva L, Valentini G, Riemekasten G, Cutolo M, Ionescu R, Czirják L, Damjanov N, Rednic S, Matucci Cerinic M (2012). ““To be or not to be,” ten years after: evidence for mixed connective tissue disease as a distinct entity”. Semin. Arthritis Rheum. 41 (4): 589–98. doi:10.1016/j.semarthrit.2011.07.010. PMID 21959290.
- ↑ Alarcón GS, Williams GV, Singer JZ, Steen VD, Clegg DO, Paulus HE, Billingsley LM, Luggen ME, Polisson RP, Willkens RF (1991). “Early undifferentiated connective tissue disease. I. Early clinical manifestation in a large cohort of patients with undifferentiated connective tissue diseases compared with cohorts of well established connective tissue disease”. J. Rheumatol. 18 (9): 1332–9. PMID 1757934.
- ↑ LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Rowell N, Wollheim F (1988). “Scleroderma (systemic sclerosis): classification, subsets and pathogenesis”. J. Rheumatol. 15 (2): 202–5. PMID 3361530.
- ↑ Roguedas AM, Misery L, Sassolas B, Le Masson G, Pennec YL, Youinou P (2004). “Cutaneous manifestations of primary Sjögren’s syndrome are underestimated”. Clin. Exp. Rheumatol. 22 (5): 632–6. PMID 15485020.
- ↑ Bablekos GD, Michaelides SA, Karachalios GN, Nicolaou IN, Batistatou AK, Charalabopoulos KA (2006). “Pericardial involvement as an atypical manifestation of giant cell arteritis: report of a clinical case and literature review”. Am. J. Med. Sci. 332 (4): 198–204. PMID 17031245.
- ↑ Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, Mispireta J, Horwitz S, Vela JE (1977). “Takayasu’s arteritis. Clinical study of 107 cases”. Am. Heart J. 93 (1): 94–103. PMID 12655.
- ↑ Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, Bienvenu B, Mouthon L, Guillevin L (2010). “Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database”. Arthritis Rheum. 62 (2): 616–26. doi:10.1002/art.27240. PMID 20112401.
- ↑ Tunç R, Uluhan A, Melikoğlu M, Ozyazgan Y, Ozdoğan H, Yazici H (2001). “A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet’s syndrome”. Clin. Exp. Rheumatol. 19 (5 Suppl 24): S45–7. PMID 11760398.
- ↑ Kunnamo I, Kallio P, Pelkonen P, Viander M (1986). “Serum-sickness-like disease is a common cause of acute arthritis in children”. Acta Paediatr Scand. 75 (6): 964–9. PMID 3564980.
- ↑ Oriente P, Biondi-Oriente C, Scarpa R (1994). “Psoriatic arthritis. Clinical manifestations”. Baillieres Clin Rheumatol. 8 (2): 277–94. PMID 8076388.
- ↑ Kaufmann J, Buccola JM, Stead W, Rowley C, Wong M, Bates CK (2007). “Secondary symptomatic parvovirus B19 infection in a healthy adult”. J Gen Intern Med. 22 (6): 877–8. doi:10.1007/s11606-007-0173-9. PMC 2219874. PMID 17384979.
Epidemiology and Demographics
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Epidemiology and Demographics
Age
It is mainly a disease of young adults (mean age, 20–30 years).
Gender
It has a slight bias towards females.
Developed Countries
Kikuchi’s disease is a very rare disease and mainly seen in Japan. Isolated cases are reported in America, Europe, and Asia.
References
Natural History, Complications and Prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Natural History
Course of the disease is generally benign and self-limiting. Lymphadenopathy most often resolves over several weeks to six months. Recurrence rate is about 3%.
Complications
Mortality is extremely rare and usually due to hepatic, respiratory, or cardiac failure.
Prognosis
- Kikuchi’s disease is a self limiting illness.
- In most reported cases, it is common for the debilitating symptoms to last for half a year; however, the disease does return to some patients after time.
References
Diagnosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Ultrasound | Other Diagnostic Studies
Treatment
Treatment
Medical Therapy | Surgery | Cost-Effectiveness of Therapy | Future or Investigational Therapies
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