Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 4194
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size

  Table of Contents    
Year : 2018  |  Volume : 61  |  Issue : 1  |  Page : 113-115
Kikuchi's disease (histiocytic necrotizing lymphadenitis): A rare presentation with acute kidney injury, peripheral neuropathy, and aseptic meningitis with cutaneous involvement

1 Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Wardha, Maharashtra, India
2 Department of Ophthalmology, Jawaharlal Nehru Medical College, Wardha, Maharashtra, India

Click here for correspondence address and email

Date of Web Publication22-Mar-2018


Kikuchi's disease (KD) also known as histiocytic necrotizing lymphadenitis is rare, idiopathic, generally self-limited cause of lymphadenitis. We present a case of twenty year young female who presented in critically ill state with fever, cervical and axillary lymphadenopathy, rash, vomiting and altered sensorium and found to have neurological, hepatic, renal and dermatological involvement. Kikuchi's disease should be considered in differential diagnosis of fever and lymphadenopathy and though benign can sometimes present with multi-organ involvement. It is because of rarity of this disease with unusual complications, present case is reported.

Keywords: Fever of unknown origin, histiocytic necrotizing lymphadenitis, Kikuchi's disease, Kikuchi-Fujimoto disease, lympadenopathy

How to cite this article:
Jain J, Banait S, Tiewsoh I, Choudhari M. Kikuchi's disease (histiocytic necrotizing lymphadenitis): A rare presentation with acute kidney injury, peripheral neuropathy, and aseptic meningitis with cutaneous involvement. Indian J Pathol Microbiol 2018;61:113-5

How to cite this URL:
Jain J, Banait S, Tiewsoh I, Choudhari M. Kikuchi's disease (histiocytic necrotizing lymphadenitis): A rare presentation with acute kidney injury, peripheral neuropathy, and aseptic meningitis with cutaneous involvement. Indian J Pathol Microbiol [serial online] 2018 [cited 2023 Oct 2];61:113-5. Available from:

   Introduction Top

Kikuchi's disease (KD) is rare, idiopathic, generally self-limited cause of lymphadenitis. It is also known as histiocytic necrotizing lymphadenitis and  Kikuchi-Fujimoto disease More Details. Kikuchi first described the disease in 1972 in Japan.[1] Fujimoto et al. independently described the disease in the same year.

The disease is usually limited to lymph nodes, but the involvement of skin, liver, spleen, and even neurological involvement and fatalities have been reported. Rare neurologic complications include aseptic meningitis, acute cerebellar ataxia, encephalitis, photophobia, mononeuritis multiplex, brachial neuritis, and hemiparesis.[2],[3] However, only a few cases of acute kidney injury (AKI), peripheral neuropathy along with aseptic meningitis and cutaneous involvement following KD have been reported.

   Case Report Top

A 20-year-old female patient presented with a history of high-grade fever, vomiting and myalgia for the past 4 days and rash over the abdomen, back, and upper limbs along with altered sensorium for 1 day. There was no history of a cough, dyspnea, chest pain, and any weakness. History of low-grade fever along with neck and axillary swellings for 4 months was present and she has received multiple courses of antibiotics during this period.

Her vital signs on admission were as follows: glasgow coma scale score was 10/15 (E2, M4, V4), pulse-118 beats/min, blood-pressure 70 mmHg systolic, febrile and had multiple, palpable, nontender lymph nodes 2 cm × 2 cm in both sides of the neck and left axilla. Petechial rashes were noted over the abdomen, back and both upper limbs [Figure 1]a, which decreased by the 5th day [Figure 1]b. On central nervous system examination, she was drowsy, and neck rigidity was present. Rest systemic examination and fundoscopic examination were normal.
Figure 1: Photograph showing petechial rashes over the abdomen, and back (a) on the day of admission (b) after 5 days

Click here to view

Pertinent laboratory data showed: hemoglobin 12.6 g/dl, total leukocyte count 14,100/cu mm, platelets 1.22 lakhs/ul, and peripheral smear showed neutrophil leukocytosis. Her urine analysis revealed albuminuria with microscopic hematuria. Renal function tests (serum creatinine-2.40 mg/dl; blood urea nitrogen-67 mg/dl) were suggestive of acute renal failure; and her ultrasound abdomen was normal. Other parameters such as blood sugar, electrolytes, arterial blood gas reports, chest X-ray, and computed tomography scan head were normal. Liver function test revealed aspartate amino transferase – 408 and alanine aminotransferase – 460 with raised lactate dehydrogenase (LDH) levels of 4822 mg/dl. She tested negative for serological tests for malaria antigen, dengue, Paul Bunnell test (heterophil agglutinins and specific Epstein-Barr virus antibodies for diagnosis of infectious mononucleosis), leptospira, and HIV. Her rheumatologic markers such as rheumatoid factor, anti-nuclear antibody, anti-double-stranded DNA, anti-perinuclear neutrophil cytoplasmic antibody were also negative.

Her lumbar puncture was done, and cerebrospinal fluid (CSF) glucose was 69 mg/dl, protein was 299 mg/dl, CSF cytology showed neutrophils. However, culture and ADA were negative and suggestive of acute aseptic meningitis.

She underwent fine-needle aspiration cytology and excision biopsy from cervical lymph nodes. Histopathology showed large paracortical, well-circumscribed necrotic lesions, with a starry-sky appearance because of tangible macrophages [Figure 2]a. There was extensive karyorrhexis, fibrin deposits, plasmacytoid monocytes, phagocytic foamy histiocytes and rare plasma cells [Figure 2]b. There were no neutrophils, no follicular hyperplasia, and no atypia. Granuloma formation was not seen. There was no arteritis. Histopathology findings revealed histiocytic necrotizing lymphadenitis suggestive of KD.
Figure 2: Microphotograph showing (a) lymph node showing large area paracortical necrosis with rim of viable areas. (H and E, ×100) (b) extensive karyorrhexis, fibrin deposition and histiocytes (H and E, ×400)

Click here to view

She was started on injectable steroids, antibiotics, and analgesics with significant improvement in her symptoms. Four days later, she developed new lesions in lower limbs in the form of palpable purpura along with bilateral wrist drop [Figure 3]a and foot drop [Figure 3]b. We suspected vasculitis to be the cause for her condition and continued on steroids. Nerve conduction study revealed severe motor plus sensory axonal neuropathy. A diagnosis of KD with aseptic meningitis with AKI and bilateral motor plus sensory axonal neuropathy was made.
Figure 3: Photograph showing both (a) wrist and (b) foot drop along with skin lesions

Click here to view

Skin biopsy could not be done as the patient was not willing for same. Clinical improvement with full recovery of sensorium and renal function was observed after 10 days. She was discharged on oral steroids and physiotherapy following which there is a gradual improvement in power of wrist and foot, but it did not improve completely when she came for follow-up after 1 month.

   Discussion Top

KD should be considered as a differential diagnosis of lymphadenitis. The course of KD is usually benign and self-limited (resolves over a period of few weeks to 6 months). The exact cause of KD is not known but considered as an autoimmune phenomenon as several authors have reported an association with viral diseases and SLE.[4]

Common manifestations of KD are cervical lymphadenopathy, rarely generalized lymphadenopathy, flu-like syndrome in almost half of the patients including fever, headache, vomiting, arthralgia, myalgia, and weight loss.[5] Skin involvement includes rash, maculopapular lesions, nodules, and urticaria.[6] Rare involvement of joints, thyroid, parotid glands, and hepatosplenomegaly with elevated levels of liver enzymes and LDH and fatal outcome from myocardial involvement has been reported.[4],[7] The patient had usual manifestations, i.e., fever, lymphadenopathy, as well as uncommon involvement in the form of rash, hepatic, renal as well as neurological involvement in the form of aseptic meningitis and peripheral neuropathy.

We ruled out close differential diagnoses of our case, i.e., lymphoma, leukemia, tuberculosis, infectious mononucleosis, SLE, rheumatoid arthritis, and also ruled out causes for her acute illness.[8]

The occurrence of peripheral neuropathy is rare in KD. We presumed that neuropathy in our patient was secondary to cryoglobulinemia, which may occur without any apparent condition or it accompanies chronic infection. In cryoglobulinemia, distal symmetrical peripheral neuropathy occurs in association with Raynaud phenomenon and purpuric eruptions of the skin. It evolves over a period of few days, more in the legs than in the arms and more common in the distribution of vascular changes as seen in our patient. The mechanisms by which cryoglobulinemia disorders causes neuropathy is uncertain, however acute vasculitic neuropathy was mentioned earlier.[9]

We treated her both with steroids and antibiotics, and she responded promptly. Immunosuppressants have been recommended in severe, life-threatening disease. However, we did not start her on cyclophosphamide as our patient responded to steroid therapy.

   Conclusion Top

It is important to remember KD as a rare cause of lymphadenopathy in young individuals. The treatment of KD is mainly supportive, and use of corticosteroids is recommended in severe extranodal or generalized KD.[10]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


The authors thank Dr. N. Gangane, Professor and Head (MD, Pathology), and Dr. S. Jain Professor (MD, Dermatology) for their contribution to the present report.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Bennie MJ, Bowles KM, Rankin SC. Necrotizing cervical lymphadenopathy caused by Kikuchi-Fujimoto disease. Br J Radiol 2003;76:656-8.  Back to cited text no. 1
Avkan-Oguz V, Yapar N, Ozakbas S, Demir-Onder K, Aktas E, Alp-Cavus S, et al. Acase of fever of unknown origin: Co-existence of Kikuchi-Fujimoto disease and acute disseminated encephalomyelitis (ADEM). Intern Med 2010;49:1823-6.  Back to cited text no. 2
Noursadeghi M, Aqel N, Pasvol G. Kikuchi's disease: A rare cause of meningitis? Clin Infect Dis 2005;41:e80-2.  Back to cited text no. 3
Dumas G, Prendki V, Haroche J, Amoura Z, Cacoub P, Galicier L, et al. Kikuchi-fujimoto disease: Retrospective study of 91 cases and review of the literature. Medicine (Baltimore) 2014;93:372-82.  Back to cited text no. 4
Parappil A, Rifaath AA, Doi SA, Pathan E, Surrun SK. Pyrexia of unknown origin: Kikuchi-Fujimoto disease. Clin Infect Dis 2004;39:138-43.  Back to cited text no. 5
Mauleón C, Valdivielso-Ramos M, Cabeza R, Rivera T, García I. Kikuchi disease with skin lesions mimicking lupus erythematosus. J Dermatol Case Rep 2012;6:82-5.  Back to cited text no. 6
Graham LE. Kikuchi-fujimoto disease and peripheral arthritis: A first! Ann Rheum Dis 2002;61:475.  Back to cited text no. 7
Primrose WJ, Napier SS, Primrose AJ. Kikuchi-fugimoto disease (Cervical subacute necrotising lymphadenitis): An important benign disease often masquerading as lymphoma. Ulster Med J 2009;78:134-6.  Back to cited text no. 8
Chad D, Pariser K, Bradley WG, Adelman LS, Pinn VW. The pathogenesis of cryoglobulinemic neuropathy. Neurology 1982;32:725-9.  Back to cited text no. 9
Yoshioka K, Miyashita T, Nakamura T, Inoue T, Yamagami K. Treatment of histiocytic necrotizing lymphadenitis (Kikuchi's disease) with prolonged fever by a single course of methylprednisolone pulse therapy without maintenance therapy: Experience with 13 cases. Intern Med 2010;49:2267-70.  Back to cited text no. 10

Correspondence Address:
Jyoti Jain
Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Sevagram, Wardha - 442102, Maharashtra
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_256_17

Rights and Permissions


  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
1 Recurrent Aseptic Meningitis Associated with Kikuchi's Disease (Histiocytic Necrotizing Lymphadenitis): A Case Report and Literature Review
Shiori Sekiguchi, Yu Yamamoto, Shuji Hatakeyama, Masami Matsumura
Internal Medicine. 2021; 60(11): 1779
[Pubmed] | [DOI]
2 Case Report: Histiocytic Necrotizing Lymphadenitis (Kikuchi–Fujimoto Disease) Concurrent With Aseptic Meningitis
Yanna Song, Shan Liu, Lei Song, Huaqiu Chen, Miaoshui Bai, Jinhua Yan, Tianfei Luo, Kangding Liu, Li Sun, Yang Zhao
Frontiers in Neurology. 2021; 12
[Pubmed] | [DOI]
3 Bibliometric Analysis of Publications from Web of Science Affiliated to Health Sciences University from 2017-2019
Roshan Umate, Manoj Patil, Shital Telrandhe, Aniket Pathade
Journal of Evolution of Medical and Dental Sciences. 2020; 9(23): 1756
[Pubmed] | [DOI]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  

   Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded97    
    Comments [Add]    
    Cited by others 3    

Recommend this journal