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Kikuchi-Fujimoto Disease

Clinical History
Examination
Investigations
Diagnosis
Treatment
Discussion
References

A 25 year male patient came with the complaints fever with chills since 3 months. Fever was high grade, intermittent and showed evening rise of temperature
He gives h/o myalgia, fatigue, loss of appetite and significant weight loss.
No h/o of cough, rashes, abdominal pain, diarrhoea.
Not a k/c/o diabetes mellitus, hypertension, thyroid disorders, cardiac disorders

Past History:
He was a k/c/o of kikuchi fujimoto disease, diagnosed 3 years back at same hospital.

General Physical Examination:

Febrile

Pallor: present

B/L cervical (posterior and anterior triangle), axillary, inguinal, and epitrochlear non-tender lymphadenopathy.

Lymph nodes were soft and non-matted.

Systemic Examination:

ABD: No organomegaly

Normal bowel sounds.

CVS: S1S2 heard. No murmurs

RS: B/L NVBS. No added sounds.

Blood Investigations:   Hb: 10.8 g% PCV: 32.4% MCHC: 33.3 g/dL TLC: 3510 cells/cumm Platelets: 1.5 lakh/cumm PBS: Microcytic hypochromic anaemia with leukopenia.  
Malaria Card Test: Immunochromatographic test – negative  
Tuberculosis Test: Acid fast bacilli (AFB) staining negative.  
Paul Bunnell Test:   Negative for infectious mononucleosis  
Fine Needle Aspiration Cytology Reactive lymphadenitis with histiocytes, tingible body macrophages.  
Biopsy: Biopsy of scalene lymph node show no granulomas but areas of necrosis and signs of lymphadenitis
 

CT FINDINGS:

2 enlarged lymph nodes showing necrotic foci and some perinodal infiltration (arrow head)

1. Kikuchi Fujimoto Disease

2. Tuberculosis

3. Infectious Mononucleosis

4. Systemic Lupus Erythematosus

TAB. PARACETAMOL 650 mg 1-1-1 PO

TAB DEXAMETHASONE 4 mg 1-1-1 PO TILL SYMPTOMS PERSIST, THEN TAPERED OVER 1 WEEK

TAB PREDNISOLONE 20 mg 1-1-0 PO TILL SYMPTOMS PERSIST, THEN TAPERED OVER 1 WEEK

TAB PANTOPRAZOLE 40 mg 1-0-0 PO

INJ. PARACETAMOL 1gm i.v SOS

Kikuchi Fujimoto is an extremely rare, sporadic and benign condition of unknown etiology which causes fever, necrotizing lymphadenopathy, rashes, headache and occasionally leukopenia[1]. The symptoms are usually self-limiting. The condition was first described in 1972 in Japan as a new type of lymphadenitis with focal reticulum cell hyperplasia[2]. This condition is almost always found in young women[3]. Over a period of 15 years (1986-2000), only 330 cases were reported worldwide[4], in contrast to the period 1996-2012 in which only 9 cases were reported[5]. This makes this case a particularly rare diagnosis.

Kikuchi disease is often associated with Systemic Lupus Erythematosus and hence must be evaluated for the same using an antinuclear antibody profile.

Clinically, the disease is often confused for tuberculosis and sarcoidosis due to similar presentation.

Histologically, necrosis, karryorhectic debris, crescent shaped histiocytes and plasmacytoid monocytes can be seen. Treatment is mainly symptomatic and relies heavily on NSAIDs and corticosteroids. Death is unlikely and there is a 3% recurrence rate.

  1. Mahajan T, Merriman R, Stone M. Kikuchi-Fujimoto Disease (Histiocytic Necrotizing Lymphadenitis): Report of a Case with Other Autoimmune Manifestations. Baylor University Medical Center Proceedings. 2007;20(2):149-151.
  2. Archibald D, Carlson M, Gustafson R. Kikuchi-Fujimoto Disease in a 30-Year-Old Caucasian Female. International Journal of Otolaryngology. 2009;2009:1-4.
  3. Deaver D, Naghashpour M, Sokol L. Kikuchi-Fujimoto Disease in the United States: Three Case Reports and Review of the Literature. Mediterranean Journal of Hematology and Infectious Diseases. 2013;6(1):e2014001.
  4. Lin H. Kikuchi’s disease: a review and analysis of 61 cases. Otolaryngology – Head and Neck Surgery. 2003;128(5):650-653.
  5. Lee B, Patel R. Kikuchi–Fujimoto Disease. Clinical Pediatrics. 2012;52(1):92-95.
  6. Image 2 – https://en.wikipedia.org/wiki/Kikuchi_disease – Copyright © 2011 Michael Bonert
  7. Image 3 – Lee S, Yoo J, Lee S. Kikuchi Disease: Differentiation from Tuberculous Lymphadenitis Based on Patterns of Nodal Necrosis on CT. 2019. American Journal of Neuroradiology January 2012, 33 (1) 135-140; DOI: https://doi.org/10.3174/ajnr.A2724

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