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BMJ Case Rep. 2010; 2010: bcr11.2009.2469.
Published online May 19, 2010. doi:  10.1136/bcr.11.2009.2469
PMCID: PMC3047526
Rare disease
Recurrent Kikuchi-Fujimoto disease
Brendan Boyd Spooner,1 Imdadur Rahman,1 Nigel Langford,1 and Robin E Ferner2
1City Hospital, Dudley Road, Birmingham, B18 7QH, UK
2City Hospital, University Department of Medicine, Dudley Road, Birmingham, B18 7QH, UK
Correspondence to Brendan Boyd Spooner, bspooner/at/doctors.org.uk
We report the case of a 27-year-old, UK-born, Asian woman who suffered a rare recurrence of Kikuchi-Fujimoto disease. She presented with tender lymphadenopathy of the neck, fever and a prodrome of flu-like symptoms. She had a past medical history of biopsy-proven Kikuchi-Fujimoto disease of her right axilla 2 years earlier, which required no treatment. Following her repeat admission, a cervical lymph node biopsy confirmed a diagnosis of recurrent Kikuchi-Fujimoto disease. She did not improve despite treatment with paracetamol and non-steroidal anti-inflammatory drugs (NSAIDs). She was started on high-dose prednisolone and her symptoms, pyrexia, and inflammatory markers settled within 24 h. She continually improved and the prednisolone was slowly reduced over 9 months. Kikuchi-Fujimoto is a rare, benign, self-limiting disease that presents with lymphadenopathy and fever. It rarely reoccurs. The severity, recurrence and the different body site make this an unusual case.
Kikuchi-Fujimoto disease (histiocytic necrotising lymphadenitis) is a rare, benign, usually self-limiting syndrome characterised by tender lymphadenopathy and fever that occurs predominantly in young people.1,2 It was first described in 1972 in Japan.1 Reoccurrence is rare and it is thought to be in the order of 3%.3 Awareness of the condition is important to prevent the disease being misdiagnosed as malignant lymphoma or tuberculosis leading to the unnecessary use of toxic agents.4 A positive diagnosis also avoids the individual from undergoing unnecessary investigations. Patients with Kikuchi-Fujimoto disease may require follow-up as there is a risk of developing systemic lupus erythematosus (SLE).5
Kikuchi-Fujimoto disease is a rare cause of tender lymphadenopathy that should be considered in young, otherwise, well individuals. Furthermore, this case illustrates that Kikuchi-Fujimoto disease can recur outside the original site.
Usually the disease is self-limiting or only requires simple analgesia or NSAIDs. If the disease fails to respond to simple measures, high-dose corticosteroids may be effective.
A 27-year-old, UK-born, Asian, female social worker presented with a 3-week history of painful swellings in the neck, fever, malaise and flu-like symptoms. Her past medical history included Kikuchi-Fujimoto disease in the right axilla (following investigations for mastalgia under a breast surgeon), asthma and migraines. She had no other systemic complaints. There was no relevant family history. She had no recent travel or contact with tuberculosis or infectious disease. She lived with her family and was a non-smoker. She had no sexual history.
On examination she had three palpable tender cervical lymph nodes in her right neck. Chest and abdominal examination were unremarkable and there were no other palpable lymph nodes. She had a temperature of 38.8°C.
Blood results showed a normal white cell count and a C reactive protein (CRP) of 8 mg/litre. The chest radiograph was normal. She was commenced on simple analgesia. Over the coming 15 days her CRP increased. She persistently had daily pyrexial episodes (>38°C) and her neck tenderness worsened.
A CT scan of her neck confirmed right-sided cervical lymphadenopathy. The ear, nose and throat team performed a cervical node biopsy on day 8. Despite the previous history of Kikuchi-Fujimoto disease, there was much debate in the pathology department about the biopsy as to whether it was lymphoma or Kikuchi-Fujimoto disease.
Her symptoms and fever continued to worsen and on day 11 she was commenced on ibuprofen when the pathologists confirmed Kikuchi-Fujimoto disease.
By day 15 there was no improvement on ibuprofen. Case reports of Kikuchi-Fujimoto disease suggested that corticosteroids might help, so 50 mg of prednisolone was started. Within 24 h her symptoms began to settle and her inflammatory markers dropped. She was discharged with a reducing dose of prednisolone 3 days later.
Increasing CRP (8 mg/litre on day 1 to 140 mg/litre on day 14); transient neutropenia (white blood cell count 3.8–10 * 109/litre, range 4–11 * 109/litre); lactate dehydrogenase 232 IU/litre (normal range <225 IU/litre); negative blood cultures × 4; autoantibody negative (anti-nuclear antibody, anti-neutrophil cytoplasmic antibody); HIV test negative; anti strep-O titre, cytomegalovirus antibodies negative; Epstein-Barr virus antibodies negative; toxoplasmosis antibodies negative; angiotensin converting enzyme negative; CT of neck: prominent right sided cervical and supraclavicular lymphadenopathy, the larger nodes having minor cystic changes; CT of thorax and abdomen/pelvis: no significant lymphadenopathy, no other abnormalities; lymph node biopsy: largest node was 20 × 10 × 5mm with patchy areas of necrosis. The features are of typical Kikuchi-Fujimoto Disease with histiocytic necrotising lymphadenitis.
Differential diagnosis
Kikuchi-Fujimoto disease, lymphoma, SLE, tuberculosis, infectious mononucleosis, Kawasaki disease, sarcoidosis, toxoplasmosis, cytomegalovirus infection, HIV, syphilis.
Treatment
Conservative management with simple analgesia and observation for 11 days. Day 11, the patient started on ibuprofen 400 mg three times a day with no improvement. Day 15 she started on prednisolone 50 mg (1 mg/kg) once a day for 1 week and then 30 mg once a day for 2 weeks. The dose was reduced over 9 months.
On day 15, CRP was 140 mg/litre. The ibuprofen had not improved the patient’s symptoms or pyrexia and 50 mg of prednisolone was started. On day 16 the patient became apyrexial and remains so. Day 17 the CRP dropped to 55 mg/litre (day 2 of prednisolone) and the patient was apyrexial. Day 19, CRP drops to 15 mg/litre (day 4 of prednisolone) and the patient was apyrexial and cervical tenderness was reducing.
At 6 weeks’ follow-up, the patient was ayprexial, CRP was <5 mg/litre and the tender cervical lymphadenopathy was improving.
At 4 months’ follow-up the patient was ayprexial, the cervical lymphadenopathy had settled and there were no signs of SLE clinically.
We conducted a Medline search for English-language reports of recurrent Kikuchi-Fujimoto disease and we found 21 cases.1,2,611 The incidence of recurrence has been reported at about 1–3%,1,3,10 but there have been more recent reports of recurrence rates as high as13% in an Asian population in Korea.6
Kikuchi-Fujimoto disease usually has a self-limiting course and does not often require treatment.3,12 When it does require treatment, simple analgesia and NSAIDs are usually sufficient.3,13,14
Systemic steroids have been used in more severe cases of Kikuchi’s disease.11,15,16 Jang and colleagues have recommended expanding the indications for corticosteroids to include recurrent disease and they used them successfully in two cases of recurrent disease in 2000.9 There are other reports of the use of oral steroids in recurrent disease.11,16
This case is unusual as the recurrence of Kikuchi-Fujimoto disease was at a different body site (cervical lymph nodes) to the first instance (axillary lymph nodes). Only one other report of recurrent disease at a different body site was found.11 Furthermore, in this case the recurrent disease was much more severe and required high-dose oral steroids, having not needed treatment in the first episode.
The favourable response to steroids in this case and others, supports the use of steroids for Kikuchi-Fujimoto disease unresponsive to other treatment modalities.9,11,15
Learning points
  • Kikcuhi-Fujimoto disease is a rare cause of persistent pyrexia and tender lymphadenopathy and can reoccur at new sites.
  • Lymph node biopsy is important in lymphadenopathy where there is diagnostic uncertainty.
  • This case reminds us of the wisdom of reviewing the patient’s previous histology.
  • Steroids are effective in relieving symptoms and reducing fever in this case of recurrent Kikuchi-Fujimoto disease, which did not respond to conservative management or NSAIDs.
Footnotes
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.
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