scholarly journals Case Report: Kikuchi-Fujimoto disease: a diagnostic and therapeutic dilemma following pretransplant nephrectomy for a 2.35 Kg kidney

F1000Research ◽  
2016 ◽  
Vol 5 ◽  
pp. 1407
Author(s):  
Arvind P. Ganpule ◽  
Jaspreet Singh Chabra ◽  
Abhishek G. Singh ◽  
Gopal R. Tak ◽  
Shailesh Soni ◽  
...  

Kikuchi-Fujimoto disease (KFD) is an extremely rare disease with a worldwide distribution and higher prevalence in Asians. It is a benign and self-limiting disorder, characterized by regional cervical lymphadenopathy accompanied with mild fever and night sweats. Lymph node histopathology is diagnostic and treating physicians should be aware of this entity as it may mimic other systemic diseases like systemic lupus erythematosus, tuberculosis, malignant lymphoma, and more rarely adenocarcinoma. Key features on lymph node biopsy are fragmentation, necrosis and karyorrhexis. Treatment includes symptomatic care, analgesics-antipyretics, corticosteroids and spontaneous recovery occurs in 1 to 4 months. We report a case of adult polycystic kidney disease (ADPKD) with end stage renal disease and episodes of fever and cervical lymphadenopathy. The infectious screen was negative and on extensive workup, the patient was found to have histiocytic-necrotizing lymphadenitis, which clinched the diagnosis of KFD.

2021 ◽  
Vol 8 (9) ◽  
pp. 1602
Author(s):  
Sushil Singla ◽  
Mohitesh Kumar ◽  
Vinod Kumar Jat ◽  
Deepika Parwan

Kikuchi-Fujimoto disease (KFD) is a rare benign condition also called histiocytic necrotizing lymphadenitis, which typically presented as fever with cervical lymphadenopathy in previously healthy individual. We presented a case of 11 year old boy with fever and cervical lymphadenopathy since 2 months. Lymph node biopsy was performed which suggested of KFD and was treated symptomatically. KFD incidence is rare but clinicians should be alert if young patient comes with fever and cervical lymphadenopathy to lower the chance of unwanted laboratory test and harmful treatment. 


2007 ◽  
Vol 86 (7) ◽  
pp. 412-413 ◽  
Author(s):  
Eimear Phelan ◽  
Emer Lang ◽  
Peter Gormley ◽  
John Lang

Cervical lymphadenopathy has many underlying etiologies. One of its rare causes is Kikuchi-Fujimoto disease (Kikuchi's disease, histiocytic necrotizing lymphadenitis). We discovered such a cause in a 37-year-old woman who had presented with malaise, night sweats, and weight loss in addition to cervical lymphadenopathy. We based our diagnosis on excisional lymph node biopsy. We also review 2 other cases of Kikuchi's disease that were diagnosed by others at our institution. Clinically and histologically, Kikuchi's disease is very similar to lymphoma, and distinguishing the two is difficult. However, despite the fact that Kikuchi's disease is benign, an accurate diagnosis is important because misdiagnosis might lead to unnecessary surgery and/or chemotherapy.


2018 ◽  
pp. bcr-2018-226457
Author(s):  
Roman Zuckerman ◽  
Louise Damiani ◽  
Hashem A Ayyad ◽  
Deborah R Alpert

We describe a 50-year-old woman with a history of thyroid cancer who presented with bilateral cervical and submandibular lymphadenopathy, low-grade fevers, and increasing fatigue. The patient underwent lymph node fine-needle aspiration, which showed no evidence of metastatic or lymphoproliferative disease. This procedure was complicated by a parapharyngeal abscess and cellulitis. She was treated unsuccessfully with various courses of antibiotics, but briefly responded to short courses of steroids. As her cervical lymphadenopathy returned, she underwent an excisional lymph node biopsy, which demonstrated caseating granulomatous lymphadenitis. Extensive review of systems, physical examination, laboratory and imaging studies demonstrated no evidence of malignancy, infection or systemic lupus erythematosus . Our patient was clinically diagnosed with Kikuchi-Fujimoto disease and successfully treated with prednisone tapered over 3 months. She remains in clinical remission.


2018 ◽  
Author(s):  
Galith Kalmi

Introduction: Kikuchi-Fujimoto’s disease (KFD) or histiocytic necrotizing lymphadenitis is a benign and self-limited disease of unknown etiology mainly affecting young women. Although the association with systemic lupus erythematosus (SLE) is well described, no case of drug-induced lupus erythematosus (DILE) associated KFD has not been reported so far. Case report: We herein report a 25-year old Caucasian woman, with no medical history and no medication except for oral estrogen-progestin contraception (levonorgestrel-ethinylestradiol), who presented with cervical lymphadenopathy, fever and arthralgia without weight loss, night sweats or skin involvement. An exhaustive infectious disease screening was negative and lymph node biopsy revealed histiocytic necrotizing lymphadenitis suggesting KFD. Autoimmune screening tests evidenced high titers of anti-histone antibodies suggesting DILE induced by estrogen-progestin medication. The patient received a short course of non-steroidal anti-inflammatory treatment for painful lymphadenitis and arthralgia. Oral levonorgestrel-ethinylestradiol contraceptive medication was stopped and KFD and DILE completely recovered with a long-term disappearance of anti-histone antibodies. Conclusion: We report the first case of KFD associated-DILE following oral levonorgestrel-ethinylestradiol medication. Even though levonorgestrel-ethinylestradiol induced lupus is well known, the association with KFD has never been reported and the physiopathology remained unknown. Keywords: Kikuchi-Fujimoto disease, Lupus, Levonorgestrel-ethinylestradiol


2004 ◽  
Vol 8 (6) ◽  
pp. 442-445
Author(s):  
Shane G. Silver ◽  
H. Chih-Ho Hong ◽  
Patricia T. Ting ◽  
Nigel J. Ball

Background: Kikuchi–Fujimoto's necrotizing lymphadenitis (KFNL) is a rare, benign, self-limited condition characterized by constitutional symptoms, lymphadenopathy, and skin lesions. Objective: We report a case of KFNL in a 43-year-old East Indian woman with a ten-year history of discoid lupus erythematosus (DLE) of the scalp and a three-month history of a erythematous plaque on the left nasal bridge, cervical lymphadenopathy, and fever. Skin biopsy samples were taken from the face and lymph node. Results: Histopathological examination of the skin revealed a mixed infiltrate of inflammatory cells, nuclear dust, and histiocytes phagocytosing nuclear debris in the reticular dermis. The lymph node showed interfollicular liquefactive necrosis, immunoblasts, and a similar cellular infiltrate as the skin. The non-necrotic areas demonstrated follicular hyperplasia. These pathological changes are associated with a diagnosis of KFNL. Conclusions: KFNL is reported in association with systemic lupus erythematosus, but only two other cases of systemic KFNL in association with DLE exist in the literature. This case is unique in that the patient presented with cutaneous and systemic KFNL in the setting of longstanding DLE.


2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Talayeh Rezayat ◽  
Matthew B. Carroll ◽  
Bryan C. Ramsey ◽  
Andria Smith

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis was first described in Japan in 1972. It is described as a benign syndrome most commonly involving cervical lymphadenopathy, fever, and night sweats. The etiology of KFD is unknown but it is thought to be triggered by an autoimmune or viral process with an exaggerated T-cell-mediated immune response. KFD can mimic other serious conditions such as lymphoma, systemic lupus erythematosus (SLE), herpes simplex, and Epstein Barr virus. Diagnosis is confirmed histopathologically. Kikuchi’s disease is typically reported to have a self-limiting course, resolving within several months and with a low recurrence rate between 3% and 4%. There is no specific treatment for KFD but any treatment is generally directed towards symptomatic relief with antipyretics and anti-inflammatory medications. In severe cases corticosteroids have been used. Here we describe a case of a previously healthy 26-year-old female that presented with fever and cervical lymphadenopathy. Malignancy and infections were ruled, and she was diagnosed with KFD histopathologically by lymph node biopsy. Her case is a severe case of KFD that despite treatment with multiple courses of corticosteroids and an immune modulating agent, relapsed.


F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 520
Author(s):  
Kevin Bryan Lo ◽  
Anna Papazoglou ◽  
Lorayne Chua ◽  
Nellowe Candelario

Kikuchi-Fujimoto disease is a form of a benign necrotizing lymphadenitis which is most commonly misdiagnosed as tuberculosis and or lymphoma, usually more common among young adults in Asia. It is a benign disease but can mimic a lot of other disease processes spanning infectious, rheumatologic and even hematologic malignancies. Our patient presented with prolonged fever and lymphadenopathy. Initial considerations were lymphoma and a nonspecific viral infection. A CT scan showed diffuse cervical lymphadenopathy with lacrimal gland involvement. An excisional lymph node biopsy was done which revealed Kikuchi disease. Patient was given steroids with immediate response with defervescence. Kikuchi is a disease with many mimics and a complete workup is needed to exclude serious disease like malignancy.


2018 ◽  
Author(s):  
Galith Kalmi

Introduction: Kikuchi-Fujimoto’s disease (KFD) or histiocytic necrotizing lymphadenitis is a benign and self-limited disease of unknown etiology mainly affecting young women. Although the association with systemic lupus erythematosus (SLE) is well described, no case of drug-induced lupus erythematosus (DILE) associated KFD has not been reported so far. Case report: We herein report a 25-year old Caucasian woman, with no medical history and no medication except for oral estrogen-progestin contraception (levonorgestrel-ethinylestradiol), who presented with cervical lymphadenopathy, fever and arthralgia without weight loss, night sweats or skin involvement. An exhaustive infectious disease screening was negative and lymph node biopsy revealed histiocytic necrotizing lymphadenitis suggesting KFD. Autoimmune screening tests evidenced high titers of anti-histone antibodies suggesting DILE induced by estrogen-progestin medication. The patient received a short course of non-steroidal anti-inflammatory treatment for painful lymphadenitis and arthralgia. Oral levonorgestrel-ethinylestradiol contraceptive medication was stopped and KFD and DILE completely recovered with a long-term disappearance of anti-histone antibodies. Conclusion: We report the first case of KFD associated-DILE following oral levonorgestrel-ethinylestradiol medication. Even though levonorgestrel-ethinylestradiol induced lupus is well known, the association with KFD has never been reported and the physiopathology remained unknown. Keywords: Kikuchi-Fujimoto disease, Lupus, Levonorgestrel-ethinylestradiol


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
K. P. Jayawickreme ◽  
S. Subasinghe ◽  
S. Weerasinghe ◽  
L. Perera ◽  
P. Dissanayaka

Abstract Background Systemic lupus erythematosus is a rare autoimmune disorder, with the prevalence in Asia ranging from 30 to 50/100,000. The diagnosis of systemic lupus erythematosus is made according to the 2019 European League Against Rheumatism/American College of Rheumatology classification criteria, and it does not contain lymphadenopathy as diagnostic criteria. However, lupus lymphadenopathy has an estimated prevalence of 5–7% at the onset of disease, and 12–15% at any stage of the disease. Case presentation A 19-year-old Sinhalese girl had neck nodules since the age of 5 years, which increased in size and became tender since 1 year. She had alopecia and joint stiffness for 6 months. She presented with a 5-day history of worsening joint pain, fever, and painful, enlarging cervical nodules. She had tender cervical lymphadenopathy, and a vasculitic rash on both lower limbs. She had pancytopenia, an erythrocyte sedimentation rate of 92, positive antinuclear antibody titer, and high anti-double-stranded deoxyribonucleic acid (DNA), with low C3 and C4 complements. She had a high reticulocyte count of 5%, with direct and indirect antiglobulin tests being positive, indicating autoimmune hemolytic anemia. Lymph node biopsy showed moderate reactive follicular hyperplasia, with scattered plasma cells and immunoblasts, with varying degree of coagulative necrosis, suggestive of lupus lymphadenopathy. On immunohistochemistry of the lymph node biopsy, Bcl2 was negative, excluding lymphoma. Contrast-enhanced computed tomography of abdomen and chest was normal with no hepatosplenomegaly or lymphadenopathy. Skin biopsy showed leukocytoclastic vasculitis. Later, with development of generalized edema, she was found to have impaired renal function, and renal biopsy showed lupus nephritis. She was started on hydroxychloroquine, prednisolone, and mycophenolate mofetil, and her symptoms improved and lymphadenopathy regressed. Conclusion In the case of cervical lymphadenopathy in a patient with systemic lupus erythematosus, the possibilities of lupus lymphadenopathy, Kikuchi–Fujimoto disease, and lymphoma should all be considered, after excluding secondary infection due to immunosuppression. Histology confirms the differentiation of these pathologies. It is important to differentiate the cause for lymphadenopathy in systemic lupus erythematosus as the outcome and treatment varies. Lupus lymphadenopathy is usually generalized, but isolated cervical lymphadenopathy could also rarely be the first presentation of systemic lupus erythematosus. Lupus lymphadenopathy can be the only presenting feature, and needs a high index in suspecting systemic lupus erythematosus, though it is not included in the diagnostic criteria.


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