15 February 2021: Articles
Unusual clinical courseAliaa Alsolami1ABCD, Rayan Altirkistani1DEF, Sarah Tayeb1ABC, Tariq Albeshri1CDE, Osama Estaitieh1AEF, Nada Rabie1DEF, Rehab Fadag2BDF, Mohammed Samannodi13ACDEF*
Am J Case Rep 2021; 22:e928760
BACKGROUND: Kikuchi-Fujimoto (KFD) disease is a rare benign syndrome of necrotizing lymphadenopathy with distinctive histologic characteristics and was first reported in 1972. It is usually manifested by painful cervical lymphadenopathy and fever in young people, predominantly in females. KFD is a self-limiting disease and recurrence is rare.
CASE REPORT: We present the case of a middle-aged woman who presented with recurrent cervical lymphadenopathy 3 times in 13 years. Diagnosis of KFD was made by excisional lymph node biopsy, showing typical histopathologic features. She was treated with oral prednisone, with a favorable response.
CONCLUSIONS: In patients with recurrent lymphadenopathy, excisional biopsy can lead to definitive diagnosis and directed treatment. KFD is an uncommon and potential cause for recurrent lymphadenopathy.
Keywords: Histiocytic Necrotizing Lymphadenitis, Histiocytosis, Sinus, Image-Guided Biopsy, Adolescent, Biopsy, Diagnosis, Differential, lymphadenopathy, Middle Aged, Recurrence
Kikuchi-Fujimoto Disease (KFD) was first reported by Kikuchi and Fujimoto in Japan in 1972. It is also known as histiocytic necrotizing lymphadenitis. Clinical features are fever and painful cervical lymphadenopathy. It is seen more commonly in females [1,2]. Diagnosis of KFD requires biopsy and histology of lymph nodes . Typical histological features of KFD include a focal proliferation of histiocytic cells and abundant nuclear debris and absence of neutrophils . Most cases resolve within 1–3 months and recurrence is rare . We present a case of undiagnosed recurrent cervical lymphadenopathy occurring 3 times in 13 years, which was found to be due to KFD.
A 52-year-old Indian woman presented with a 7-day history of fever and painful right-sided cervical lymphadenopathy. The review of symptoms was otherwise unremarkable. The patient reported 2 similar episodes of self-limiting cervical lymphadenopathy and fever, occurring 7 and 13 years ago. She did not seek medical care on either occasion.
The patient was febrile (38.5°C) and other vital signs were within normal limits. Results of a physical examination were notable only for right-sided tender and firm cervical lymphadenopathy.
A complete blood count showed white blood cell count 7200/ cmm, hemoglobin 12.6 g/dl, and platelets 140 000/cmm. She was found to have BUN 5.3 mmol/l, serum creatinine 60 umol/l, serum ferritin 500 ug/l, and C-reactive protein (CRP) 60 mg/l. Nasopharyngeal SARS-COV2 PCR was negative. Epstein-Barr viral antibodies, HIV, rheumatoid factor, and antinuclear antibodies (ANA) were negative.
A blood culture was sent and broad-spectrum antibiotics were started empirically. A neck ultrasound revealed multiple cervical lymph nodes; the largest was 1.9×0.9 cm on the right side. CT neck and chest showed right-sided multiple cervical lymph nodes; the largest was 2.8×2.3 cm (Figure 1).
The patient continued to be febrile despite treatment with broad-spectrum antibiotics. On day 3 of hospitalization, she underwent right cervical lymph node excisional biopsy, which showed nuclear debris and necrosis with infiltration of many histiocytic cells, and no neutrophils were seen (Figure 2). Lymph node bacterial, fungal, and tuberculosis cultures were negative. Based on pathology results, a diagnosis of KFD was established.
Broad-spectrum antibiotics were stopped and oral naproxen was initiated. Due to lack of response to oral naproxen for 3 days, we switched it to oral prednisone. After 3 days of oral prednisone, her fever subsided, with near complete resolution of right cervical lymphadenopathy. She was discharged home on a short course of oral prednisone. Outpatient follow-up assessment 1 week after discharge revealed complete resolution of cervical lymphadenopathy.
KFD is a rare idiopathic cause of lymphadenopathy, first reported in 1972. The disease commonly presents with unilateral cervical lymphadenopathy in young people . The size of lymph nodes is usually 1–4 cm, but up to 6 cm has been reported [6,7]. In our case, the size of the swollen lymph node was 2.8 cm.
Excisional lymph node biopsy is the preferred diagnostic method, as the disease may affect the lymph node partially. Typical histopathological features can distinguish KFD from other diseases. These features were seen in our case and confirmed the diagnosis.
Treatment of KFD is generally supportive and patients usually respond to non-steroidal anti-inflammatory drugs (NSAID). Similar to our case, corticosteroids have induced good response in patients who did not improve with NSAID [8,9].
Recurrence of disease seems to be rare and is reported in no more than 2–3% of patients. Our patient presented with recurrent symptoms 3 times over a period of 13 years. Her first and second episodes spontaneously resolved, but the current episode needed corticosteroid therapy to induce remission.
In recurrent febrile unilateral lymphadenopathy, excisional biopsy can lead to a definitive diagnosis and directed treatment. Recurrence of KFD is rare. Therefore, long-term follow-up of patients with KFD is needed.
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2.. Jung IY, Ann HW, Kim JJ, The incidence and clinical characteristics by gender differences in patients with Kikuchi-Fujimoto disease: Medicine (Baltimore), 2017; 96(11); e6332
3.. Masab M, Surmachevska N, Kikuchi disease: StatPearls Jun 29, 2020; 2020, Treasure Island (FL), StatPearls Publishing
4.. Rezkalla J, Lynch DW, A rare case of lymphadenopathy: Kikuchi-Fujimoto disease: S D Med, 2017; 70(7); 311-13
5.. Perry AM, Choi SM, Kikuchi-Fujimoto disease: A review: Arch Pathol Lab Med, 2018; 142(11); 1341-46
6.. Dorfman RF, Berry GJ, Kikuchi’s histiocytic necrotizing lymphadenitis: An analysis of 108 cases with emphasis on differential diagnosis: Semin Diagn Pathol, 1988; 5; 329-45
7.. Lin HC, Su CY, Huang SC, Kikuchi’s disease in Asian children: Pediatrics, 2005; 115(1); e92-96
8.. Gerwig U, Weidmann RG, Lindner G, Relapsing Kikuchi-Fujimoto disease requiring prolonged steroid therapy: Case Rep Emerg Med, 2019; 2019; 6405687
9.. Jang YJ, Park KH, Seok HJ, Management of Kikuchi’s disease using glucocorticoids: J Laryngol Otol, 2000; 114; 709-11
10.. Song JY, Lee J, Park DW, Clinical outcome and predictive factors of recurrence among patients with Kikuchi’s disease: Int J Infect Dis, 2009; 13; 322-26
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