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Motwani J, Kumar A, Azhar L, Qureshi AA, Fatima Z, Khalid S, Kumari V, Mahmud Nishat S. Diagnostic challenges and management of kikuchi-fujimoto disease: a rare case report. Ann Med Surg (Lond) 2025; 87:403-406. [PMID: 40109630 PMCID: PMC11918722 DOI: 10.1097/ms9.0000000000002861] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/10/2024] [Accepted: 12/02/2024] [Indexed: 03/22/2025] Open
Abstract
Introduction Kikuchi-Fujimoto disease, otherwise referred to as histiocytic necrotizing lymphadenitis, is a rare and self-limiting disorder characterized by fever, lymphadenopathy, and upper respiratory symptoms. The main target of the illness is young Asian adults and is more prevalent in females. This disease probably has viral triggering or autoimmune responses, and diagnosis depends on histopathological examination. Case Presentation We present a case of an 18-month history of recurrent high-grade fever with marked weight loss and an enlarged occipital lymph node in a 42-year-old diabetic male from Karachi, Pakistan. The patient's symptoms did not subside despite being hospitalized several times and receiving multiple courses of antibiotics. Physical examination revealed a firm, non-tender occipital lymph node. Laboratory studies showed mild anemia and leukopenia, raised inflammatory markers, but unremarkable imaging studies and autoimmune tests. An excisional biopsy of the lymph node confirmed KFD with necrotizing lymphadenitis showing characteristic karyorrhectic debris. Discussion This case epitomizes the diagnostic challenges of KFD, which can mimic a host of conditions like tuberculosis and lymphoma. The recurrent fevers in the presence of significant weight loss and failure to respond to the usual treatments raised suspicion for further detailed investigation. Excisional biopsy established the diagnosis and differentiated it from other conditions. The patient's rapid improvement with corticosteroid therapy aligns with established treatment protocols for KFD, thus proving its efficacy. Conclusion KFD should be included in the differential diagnosis of fever of unknown origin. Diagnosis and management of KFD can avoid misdiagnosis and hence improve patient outcomes.
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Affiliation(s)
- Jatin Motwani
- Department of Internal Medicine, Liaquat National Hospital and Medical College, Karachi, Pakistan
| | - Ameet Kumar
- Department of Internal Medicine, Liaquat National Hospital and Medical College, Karachi, Pakistan
| | - Laiba Azhar
- Department of Internal Medicine, Liaquat National Hospital and Medical College, Karachi, Pakistan
| | - Ayaan Ahmed Qureshi
- Department of Internal Medicine, Liaquat National Hospital and Medical College, Karachi, Pakistan
| | - Zukhruf Fatima
- Department of Internal Medicine, Kind Edward Medical University, Lahore, Pakistan
| | - Saif Khalid
- Department of Internal Medicine, Royal College of Surgeons in Ireland, Dublin, Ireland
| | - Verkha Kumari
- Department of Internal Medicine, Liaquat National Hospital and Medical College, Karachi, Pakistan
| | - Syeed Mahmud Nishat
- Department of Internal Medicine, Shaheed Suhrawardy Medical College, Dhaka, Bangladesh
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2
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Anini EM, AlBaik TM, Ibrahim AT, AbuKaresh NA, Albandak M, Alshalaldeh R, Natsheh M, Abunejma FM. Kikuchi-Fujimoto Disease: A Case Report of Prolonged Fever and Lymphadenopathy in a Young Girl. J Investig Med High Impact Case Rep 2024; 12:23247096241246618. [PMID: 38605558 PMCID: PMC11010749 DOI: 10.1177/23247096241246618] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/04/2024] [Revised: 02/13/2024] [Accepted: 03/22/2024] [Indexed: 04/13/2024] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disorder that usually presents with regional cervical lymphadenopathy and fever. We report a case of a 12-year-old female who complained of fever, night sweating, significant weight loss, and tender right cervical lymph node enlargement for 2 months. A full workup including laboratory tests and imaging studies, an excisional biopsy, and histopathological analysis were done, and the diagnosis of KFD was confirmed. The patient was treated with analgesia and oral prednisolone, resulting in good improvement. A high degree of clinical suspicion is imperative for physicians, given the rarity of the disease and the associated diagnostic challenges.
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Yousefi M, Rukerd MRZ, Binafar H, Shoaie S, Mirkamali H, Pourzand P, Kaveh R. The co-occurrence of Kikuchi-Fujimoto disease and systemic lupus erythematosus: a case report. J Med Case Rep 2023; 17:448. [PMID: 37884991 PMCID: PMC10605312 DOI: 10.1186/s13256-023-04186-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/22/2023] [Accepted: 09/20/2023] [Indexed: 10/28/2023] Open
Abstract
BACKGROUND Kikuchi-Fujimoto disease is an uncommon systemic disease that mostly affects young women. Kikuchi-Fujimoto disease typically manifests as necrotizing lymphadenopathy, which frequently follows by a fever; however, Kikuchi-Fujimoto disease occurs rarely in extranodal regions. One of the most important accompaniments of Kikuchi-Fujimoto disease is its connection with autoimmune diseases such as systemic lupus erythematosus. This case presents a simultaneous occurrence of Kikuchi-Fujimoto disease with liver involvement and systemic lupus erythematosus in a young female patient. CASE PRESENTATION We present a rare case of a 20-year-old white woman who presented with fever, joint pains, myalgia, and shortness of breath. Initial hospitalization and treatment for fever of unknown origin did not yield improvement. Physical examination revealed cervical and supraclavicular lymphadenopathy, and laboratory investigations showed abnormal blood counts, elevated inflammatory markers, and positive autoimmune serologies. Imaging studies revealed bilateral pleural effusion and liver lesions. Lymph node biopsy confirmed the diagnosis of Kikuchi-Fujimoto disease, and liver biopsy showed extranodal involvement. The patient was diagnosed with Kikuchi-Fujimoto disease-associated systemic lupus erythematosus and treated with hydroxychloroquine and corticosteroids. The patient showed gradual resolution of symptoms and lymphadenopathy with treatment. CONCLUSION Kikuchi-Fujimoto disease is a rare systemic condition primarily impacting young females. It is characterized by necrotizing lymphadenopathy, often accompanied by fever. Although Kikuchi-Fujimoto disease is predominantly seen in the lymph nodes, occurrences in non-nodal areas are infrequent. When diagnosing Kikuchi-Fujimoto disease, it is essential to screen patients for systemic lupus erythematosus. In this particular case, we observed liver involvement along with the presence of both Kikuchi-Fujimoto disease and systemic lupus erythematosus.
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Affiliation(s)
- Maysam Yousefi
- Infectious Diseases Research Center of Tropical and Infectious Diseases, Kerman University of Medical Sciences, Kerman, Iran
| | - Mohammad Rezaei Zadeh Rukerd
- Gastroenterology and Hepatology Research Center, Institute of Basic and Clinical Physiology Sciences, Kerman University of Medical Sciences, Kerman, Iran
| | - Hanieh Binafar
- Student Research Committee, School of Medicine, Kerman University of Medical Sciences, Kerman, Iran
| | - Sahar Shoaie
- Department of Internal Medicine, Afzalipour Hospital, Kerman University of Medical Sciences, Kerman, Iran
| | - Hanieh Mirkamali
- Student Research Committee, School of Medicine, Kerman University of Medical Sciences, Kerman, Iran
| | - Pouria Pourzand
- Department of Emergency Medicine, School of Medicine, University of Medicine, Minneapolis, USA
| | - Roxana Kaveh
- Student Research Committee, School of Medicine, Kerman University of Medical Sciences, Kerman, Iran.
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4
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Nair PR, Bankar NJ, Choudhary A, Shrivastava D. Kikuchi Fujimoto Lymphadenitis: A Rare Association With COVID-19 Vaccination. Cureus 2023; 15:e45979. [PMID: 37900436 PMCID: PMC10600953 DOI: 10.7759/cureus.45979] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/17/2023] [Accepted: 09/25/2023] [Indexed: 10/31/2023] Open
Abstract
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon cause of protracted cervical lymphadenopathy in both children and adults. Although the majority of cases have been documented in Asia, this disease has been characterized globally since it was first identified in Japan in 1972. KFD's etiology is not entirely understood, although various theories have been postulated. Usually benign, Kikuchi-Fujimoto disease resolves within six months. KFD must be distinguished from other causes of chronic lymphadenopathy, such as lymphoma, inflammatory illnesses, autoimmune conditions, viral lymphadenopathy, and also from tuberculosis, especially in India, where it is still endemic. Here, we present the case report of a healthcare worker with a known case of hypothyroidism and autoimmune skin disorder who presented with prolonged fever, joint pain, and generalized lymphadenopathy post-COVID vaccination and was diagnosed as having KFD on biopsy, which can be associated with a very unusual presentation of this disease.
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Affiliation(s)
- Priya R Nair
- Obstetrics and Gynaecology, Datta Meghe Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
| | - Nandkishor J Bankar
- Microbiology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
| | - Amruta Choudhary
- Obstetrics and Gynaecology, Datta Meghe Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
| | - Deepti Shrivastava
- Obstetrics and Gynaecology, Datta Meghe Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
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Mahajan VK, Sharma V, Sharma N, Rani R. Kikuchi-Fujimoto disease: A comprehensive review. World J Clin Cases 2023; 11:3664-3679. [PMID: 37383134 PMCID: PMC10294163 DOI: 10.12998/wjcc.v11.i16.3664] [Citation(s) in RCA: 19] [Impact Index Per Article: 9.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/27/2022] [Revised: 03/29/2023] [Accepted: 04/27/2023] [Indexed: 06/02/2023] Open
Abstract
Kikuchi-Fujimoto disease, a rare form of necrotizing lymphadenitis, is an uncommon, benign, self-limiting disorder of obscure etiology. It affects mostly young adults of both genders. Clinically, it presents with fever and lymphadenopathy of a firm to rubbery consistency frequently involving cervical lymph nodes while weight loss, splenomegaly, leucopenia, and elevated erythrocyte sedimentation rate feature in severely affected patients. Cutaneous involvement occurs in about 30%-40% of cases as facial erythema and nonspecific erythematous papules, plaques, acneiform or morbilliform lesions of great histologic heterogeneity. Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an obscure and complex relationship as systemic lupus erythematosus may occasionally precede, develop subsequently, or sometimes be associated concurrently with Kikuchi-Fujimoto disease. It is often mistaken for non-Hodgkin lymphoma while lupus lymphadenitis, cat-scratch disease, Sweet's syndrome, Still's disease, drug eruptions, infectious mononucleosis, and viral or tubercular lymphadenitis are other common differentials. Fine needle aspiration cytology mostly has features of nonspecific reactive lymphadenitis and immunohistochemistry studies usually show variable features of uncertain diagnostic value. Since its diagnosis is exclusively from histopathology, it needs to be evaluated more carefully; an early lymph node biopsy will obviate the need for unnecessary investigations and therapeutic trials. Its treatment with systemic corticosteroids, hydroxychloroquine, or antimicrobial agents mostly remains empirical. The article reviews clinicoepidemiological, diagnostic, and management aspects of KFD from the perspective of practicing clinicians.
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Affiliation(s)
- Vikram K Mahajan
- Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
| | - Vikas Sharma
- Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
| | - Neeraj Sharma
- Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
| | - Ritu Rani
- Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
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Fadul A, Subahi EA, Ali EA, Awadalkareem H, Mohamed G, Elawad M, Sharaf Eldean M, Albozom A. Kikuchi-Fujimoto Disease: A Rare Cause of Pyrexia of Unknown Origin and Cervical Lymphadenopathy. Cureus 2022; 14:e30823. [DOI: 10.7759/cureus.30823] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/29/2022] [Indexed: 11/06/2022] Open
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Kikuchi-Fujimoto Disease: A Differential for When It is Not Systemic Lupus Erythematosus. Case Rep Rheumatol 2022; 2022:7709246. [PMID: 35968155 PMCID: PMC9363934 DOI: 10.1155/2022/7709246] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/07/2022] [Revised: 06/30/2022] [Accepted: 07/11/2022] [Indexed: 11/23/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare and benign disease process that is characterized by fever and lymphadenopathy that was first described in young Japanese women in the early 1970s. Knowledge of KFD is important as it can often mimic other causes of lymphadenopathy including systemic lupus erythematosus (SLE) or malignancies, and this can lead to invasive diagnostic testing and even treatments that can be avoided. The etiology and exact mechanism by which KFD develops is not fully understood at this time, but is thought to be an immune response of T cells and histiocytes to viral or bacterial infections. We present a 35-year-old African-American woman who was referred to the rheumatology clinic by our colleagues in the breast clinic with new onset right axillary lymphadenopathy and abnormal serologic testing with the suspicion of SLE after a malignancy had been ruled out.
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Zheng Y, Du Y, Zhu WH, Zhao CG. Clinical Analysis of 44 Children with Subacute Necrotizing Lymphadenitis. Infect Drug Resist 2022; 15:1449-1457. [PMID: 35392366 PMCID: PMC8979771 DOI: 10.2147/idr.s351191] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2021] [Accepted: 03/11/2022] [Indexed: 11/23/2022] Open
Abstract
Objective Methods Results Conclusion
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Affiliation(s)
- Yue Zheng
- Department of Pediatrics, Shengjing Hospital of China Medical University, Shenyang, 111004, People’s Republic of China
| | - Yue Du
- Department of Pediatrics, Shengjing Hospital of China Medical University, Shenyang, 111004, People’s Republic of China
| | - Wan-Hong Zhu
- Department of Pediatrics, Shengjing Hospital of China Medical University, Shenyang, 111004, People’s Republic of China
| | - Cheng-Guang Zhao
- Department of Pediatrics, Shengjing Hospital of China Medical University, Shenyang, 111004, People’s Republic of China
- Correspondence: Cheng-Guang Zhao, Department of Pediatrics, Shengjing Hospital of China Medical University, Shenyang, 110004, People’s Republic of China, Tel +8618940255157, Email
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Al Manasra AR, Al-Domaidat H, Aideh MA, Al Qaoud D, Al Shalakhti M, Al Khatib S, Fataftah J, Al-Taher R, Nofal M. Kikuchi-Fujimoto disease in the Eastern Mediterranean zone. Sci Rep 2022; 12:2703. [PMID: 35177750 PMCID: PMC8854556 DOI: 10.1038/s41598-022-06757-9] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2021] [Accepted: 02/02/2022] [Indexed: 11/17/2022] Open
Abstract
Kikuchi–Fujimoto disease (KFD) is a rare benign and self-limiting syndrome. We aim to review cases of KFD at our institution as a rare illness in the Arab ethnic descent and to analyse reports from most countries in the East Mediterranean zone. This is a retrospective study in which the histopathology database was searched for the diagnosis of KFD. A full review of KFD patients’ medical records was done. Data regarding demographic features, clinical presentation, laboratory findings, comorbidities, and management protocols were obtained. Published KFD cases from east Mediterranean countries were discussed and compared to other parts of the world. Out of 1968 lymph node biopsies studied, 11 (0.6%) cases of KFD were identified. The mean age of patients with KFD was 32 years (4–59). 73% (8/11) were females. The disease was self-limiting in 5 patients (45%); corticosteroid therapy was needed in 4 patients (34%). One patient was treated with methotrexate and one with antibiotics. One patient died as a consequence of lymphoma. Jordanians and Mediterranean populations, especially those of Arab ethnic background, seem to have low rates of KFD. The genetic susceptibility theory may help to explain the significantly higher disease prevalence among East Asians. Early diagnosis of KFD—although challenging—is essential to reduce the morbidity related to this illness.
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Affiliation(s)
- Abdel Rahman Al Manasra
- Department of General Surgery and Urology, Faculty of Medicine, Jordan University of Science and Technology, P.O. Box 3030, Irbid, Jordan.
| | - Hamzeh Al-Domaidat
- Department of General Surgery and Urology, Faculty of Medicine, Jordan University of Science and Technology, P.O. Box 3030, Irbid, Jordan
| | - Mohd Asim Aideh
- Department of General Surgery and Urology, Faculty of Medicine, Jordan University of Science and Technology, P.O. Box 3030, Irbid, Jordan
| | - Doaa Al Qaoud
- Department of Pediatrics, Faculty of Medicine, The Hashemite University, Zarqa, Jordan
| | - Majd Al Shalakhti
- Department of General Surgery and Urology, Faculty of Medicine, Jordan University of Science and Technology, P.O. Box 3030, Irbid, Jordan
| | - Sohaib Al Khatib
- Department of Pathology, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan
| | - Jehad Fataftah
- Department of Radiology, Faculty of Medicine, The Hashemite University, Zarqa, Jordan
| | - Raed Al-Taher
- Department of Surgery, Faculty of Medicine, The University of Jordan, Amman, Jordan
| | - Mohammad Nofal
- Department of General Surgery and Urology, Faculty of Medicine, Jordan University of Science and Technology, P.O. Box 3030, Irbid, Jordan
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YILDIRIM S, AŞIK A, ÇOBANOĞLU ŞİMŞEK B, BOZKURT A. Case report: Kikuchi-Fujimoto disease or lymphoma? KAHRAMANMARAŞ SÜTÇÜ İMAM ÜNIVERSITESI TIP FAKÜLTESI DERGISI 2021. [DOI: 10.17517/ksutfd.900042] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022] Open
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Akhavanrezayat A, Cooper JD, Hassan M, Pham BH, Nguyen QD, Farr AK. Bilateral preretinal hemorrhage associated with Kikuchi-Fujimoto disease. Am J Ophthalmol Case Rep 2021; 22:101041. [PMID: 33732946 PMCID: PMC7940994 DOI: 10.1016/j.ajoc.2021.101041] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/04/2021] [Revised: 02/03/2021] [Accepted: 02/20/2021] [Indexed: 11/19/2022] Open
Abstract
Purpose To present a case of a patient with human immunodeficiency virus (HIV) disease and Kikuchi-Fujimoto disease (KFD) who presented with a unique pattern of retinopathy. Observations A 7-year-old Taiwanese girl with HIV disease who was recently diagnosed with KFD had a sudden onset of blurry vision in both eyes one month after her KFD systemic symptoms had relatively resolved. Ophthalmic examination showed decreased visual acuity in both eyes (OU). On fundus examination, she had bilateral preretinal, subhyaloid, and vitreous hemorrhage that was more severe than anemic retinopathy. Conclusion Ocular manifestations in Kikuchi-Fujimoto disease are rare; however, if they occur, presentations may vary. The exact etiology of the disease has remained elusive and controversial. This case is the first report of a patient with HIV disease and KFD presenting with ocular involvement. Furthermore, bilateral preretinal, subhyaloid, and vitreous hemorrhage, which was beyond anemic retinopathy, is an unprecedented manifestation of KFD that has not been previously reported. This case highlights the necessity for clinicians to consider all possible differential diagnoses when evaluating patients with similar findings to identify the best therapeutic approach and avoid unnecessary treatment.
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Affiliation(s)
- Amir Akhavanrezayat
- Byers Eye Institute, Stanford University School of Medicine, Palo Alto, CA, USA
| | - Joseph D. Cooper
- Division of AIDS Medicine & Infectious Diseases, Department of Medicine, Santa Clara Valley Health & Hospital System, San Jose, CA, USA
| | - Muhammad Hassan
- Byers Eye Institute, Stanford University School of Medicine, Palo Alto, CA, USA
| | - Brandon H. Pham
- Byers Eye Institute, Stanford University School of Medicine, Palo Alto, CA, USA
| | - Quan Dong Nguyen
- Byers Eye Institute, Stanford University School of Medicine, Palo Alto, CA, USA
| | - Arman K. Farr
- Retina Institute of The Carolina and Macular Degeneration Center, Charlotte, NC, USA
- Corresponding author.
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Kikuchi-Fujimoto disease associated with primary Sjögren's syndrome - literature review based on a case report. Reumatologia 2020; 58:251-256. [PMID: 32921833 PMCID: PMC7477469 DOI: 10.5114/reum.2020.98438] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2020] [Accepted: 08/04/2020] [Indexed: 12/17/2022] Open
Abstract
Primary Sjögren’s syndrome (pSS) is a chronic, autoimmune disease predominantly involving exocrine glands. Lymphadenopathy is one of the possible symptoms of pSS. It may also suggest development of non-Hodgkin lymphoma (NHL), the most severe complication of pSS, or be a symptom of less common diseases, such as Kikuchi-Fujimoto disease (KFD), presented in this paper. Kikuchi-Fujimoto disease is an extremely rare, benign and self-limiting disorder, characterized by regional lymphadenopathy. This paper presents a case of previously unreported association of pSS, KFD and renal cancer in a patient with recurrent cervical lymphadenopathy, as well as a discussion on the coexistence of these diseases based on available literature searching for PubMed, Scopus and Google Scholar databases, particularly in this subject. These three clinical entities may manifest lymphadenopathy each, causing a diagnostic dilemma. The treatment is also challenging under such circumstances. In this particular situation, it was a combination of immunosuppressive therapy and surgery.
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Chong Y, Lee JY, Thakur N, Kang CS, Lee EJ. Strong association of Torque teno virus/Torque teno-like minivirus to Kikuchi-Fujimoto lymphadenitis (histiocytic necrotizing lymphadenitis) on quantitative analysis. Clin Rheumatol 2020; 39:925-931. [PMID: 31782015 DOI: 10.1007/s10067-019-04851-4] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2019] [Revised: 09/06/2019] [Accepted: 11/08/2019] [Indexed: 12/15/2022]
Abstract
INTRODUCTION Kikuchi-Fujimoto disease (KFD) is a rare benign lymphadenitis that mainly involves cervical lymph nodes of young Asian women with unknown etiology. Recently, we experienced a case of KFD found with Torque teno virus/Torque teno-like mini virus (TTV/TTMV) from a 26-year-old woman. TTV/TTMV is a genus of Circoviridae that causes necrotizing lymphadenitis in pigs, which shares the key histologic finding of KFD. The purpose of this study is to investigate the pathogenic role of TTV/TTMV in KFD by quantitative polymerase chain reaction (qPCR) analysis. METHOD We performed two-step qPCR specific to TTV/TTMV with formalin-fixed paraffin-embedded tissue of sequentially selected 100 KFD patients and 50 randomly selected, matched normal controls. Consequent direct sequencing was done for confirmation with PCR products. RESULTS PCR amplification of TTV and TTMV was found in a significantly higher proportion in KFDs than normal controls (TTV, 85% vs. 18%, p < 0.000; TTMV, 91% vs. 24%, p < 0.000). After the sequencing, KFD samples showed more sequence matching than control samples for TTMV (94% vs. 30%, p < 0.000). CONCLUSION This finding strongly suggests the possible implication of TTV/TTMV in the pathogenesis of KFD. Animal or in vivo experimental design should be followed in the future.Key Points• Kikuchi-Fujimoto disease (KFD) is rare and its etiology is still unclear.• Torque teno/Torque teno-like minivirus (TTV/TTMV) is a recently introduced virus in the Circoviridae family that causes necrotizing lymphadenitis in pigs, histologically similar to KFD.• We discovered the significantly increased TTV/TTMV viral loads in the KFD patients than normal controls, which implicates TTV/TTMV in the pathogenesis of KFD.
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Affiliation(s)
- Yosep Chong
- Department of Hospital Pathology, Yeouido St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 10, 63-ro, Yeongdeungpo-gu, Seoul, 07345, Republic of Korea.
| | - Ji Young Lee
- Department of Hospital Pathology, Yeouido St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 10, 63-ro, Yeongdeungpo-gu, Seoul, 07345, Republic of Korea
| | - Nishant Thakur
- Department of Hospital Pathology, Yeouido St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 10, 63-ro, Yeongdeungpo-gu, Seoul, 07345, Republic of Korea
| | - Chang Suk Kang
- Department of Hospital Pathology, Yeouido St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 10, 63-ro, Yeongdeungpo-gu, Seoul, 07345, Republic of Korea
- Department of Pathology, Samkwang Medical Laboratories, 57, Baumoe-ro 41-gil, Seocho-gu, Seoul, 06742, Republic of Korea
| | - Eun Jung Lee
- Department of Hospital Pathology, Yeouido St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 10, 63-ro, Yeongdeungpo-gu, Seoul, 07345, Republic of Korea
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14
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Singh JM, Shermetaro CB. Kikuchi-Fujimoto Disease in Michigan: A Rare Case Report and Review of the Literature. CLINICAL MEDICINE INSIGHTS. EAR, NOSE AND THROAT 2019; 12:1179550619828680. [PMID: 30833818 PMCID: PMC6393831 DOI: 10.1177/1179550619828680] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 12/10/2018] [Accepted: 12/28/2018] [Indexed: 11/16/2022]
Abstract
Background: Kikuchi-Fujimoto Disease (KFD), also known as Histiocytic Necrotizing Lymphadenitis, is a rare cause of prolonged cervical lymphadenopathy in both the pediatric and adult populations. It was first reported in Japan in 1972, and since, this disease has been described worldwide, although most cases have been reported in Asia. The etiology of KFD is not fully understood, although there are 2 theories that are described in detail in this review. Kikuchi-Fujimoto Disease typically follows a benign course, with resolution of the lymphadenopathy within 6 months. It is important to recognize KFD as a cause of persistent lymphadenopathy, as it shares many characteristics with and must be differentiated from other causes of lymphadenopathy, including lymphoma, inflammatory disorders, autoimmune conditions, and infectious causes of lymphadenopathy. Case presentation: Here is presented a case of an 11-year-old male who was born in Vietnam, but subsequently adopted and raised in the United States, who presented to a private practice community-based Otolaryngology group. His chief complaint was a persistent neck mass of approximately 3 months duration. He underwent excisional biopsy for suspected lymphoma, but final pathology rendered a diagnosis of KFD. Conclusion: The purpose of this article is not only to review the literature but also to contribute awareness of this entity in the differential diagnosis of persistent lymphadenopathy, especially for the general Otolaryngologist in a community-based setting. In addition, this review would be beneficial for other practitioners as well, specifically Pediatricians, Infectious Disease Physicians, Rheumatologists, Pathologists, and Medical Oncologists.
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Affiliation(s)
- Jeffrey Michael Singh
- Department of Otolaryngology/Head and Neck Surgery, McLaren Oakland Hospital, Pontiac, MI, USA
- Jeffrey Michael Singh, McLaren Oakland Hospital, 50 North Perry St, Pontiac, IL 48342, USA.
| | - Carl Bernard Shermetaro
- Department of Otolaryngology/Head and Neck Surgery, McLaren Oakland Hospital, Pontiac, MI, USA
- North Oakland Ear, Nose & Throat Centers, P.C., Clarkston, MI, USA
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15
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Mathew LM, Kapila R, Schwartz RA. Kikuchi-Fujimoto disease: a diagnostic dilemma. Int J Dermatol 2016; 55:1069-75. [PMID: 27207311 DOI: 10.1111/ijd.13314] [Citation(s) in RCA: 29] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/18/2015] [Revised: 12/08/2015] [Accepted: 02/03/2016] [Indexed: 11/30/2022]
Abstract
Kikuchi-Fujimoto disease is a benign, self-limiting condition characterized by lymphadenopathy and fever. Its diagnosis can be challenging, as the disease must be differentiated from lymphoma and systemic lupus erythematosus. Patients can present with nonspecific cutaneous lesions. Adequate clinical and histopathologic correlation can aid in the timely diagnosis of Kikuchi's disease, thus preventing the use of unnecessary diagnostic procedures and inappropriate treatments.
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Affiliation(s)
- Laju M Mathew
- Dermatology, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA
| | - Rajendra Kapila
- Infectious Diseases, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA.,Medicine, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA
| | - Robert A Schwartz
- Dermatology, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA. .,Medicine, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA. .,Pathology, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA. .,Pediatrics, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA. .,Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA.
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16
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Deaver D, Horna P, Cualing H, Sokol L. Pathogenesis, diagnosis, and management of Kikuchi-Fujimoto disease. Cancer Control 2015; 21:313-21. [PMID: 25310212 DOI: 10.1177/107327481402100407] [Citation(s) in RCA: 69] [Impact Index Per Article: 6.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
BACKGROUND Kikuchi-Fujimoto disease (KFD) is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. This disease is misdiagnosed as malignant lymphoma in up to one-third of cases and is associated with the development of systemic lupus erythematosus (SLE). METHODS The medical literature between the years 1972 and 2014 was searched for KFD, and the data were collected and analyzed regarding the epidemiology, clinical presentations, diagnosis, management, and suggested diagnostic and treatment algorithms. RESULTS Although KFD has been reported in other ethnic groups and geographical areas, it is more frequently diagnosed in young women of Asian descent. Patients with the disease typically present with rapidly evolving tender cervical lymphadenopathy, night sweats, fevers, and headache. Diagnosis is based on histopathological examination. Excisional lymph node biopsy is essential for a correct diagnosis. Apoptotic coagulation necrosis with karyorrhectic debris and the proliferation of histiocytes, plasmacytoid dendritic cells, and CD8(+) T cells in the absence of neutrophils are characteristic cytomorphology features. Interface dermatitis at the onset of KFD may be a marker for the subsequent evolution of SLE. The natural course of the disease is typically benign. Short courses of steroids, nonsteroidal anti-inflammatory drugs, or hydroxychloroquine can be administered to patients with more severe symptoms. CONCLUSIONS Although KFD was described more than 40 years ago, the etiology of this disease remains unsolved. Infectious or autoimmune processes were proposed but have not been definitively confirmed. Clinical presentation with systemic B symptoms and adenopathy may lead to an erroneous diagnosis of malignant lymphoma. The introduction of modern methods into hematopathology, including immunohistochemistry, flow cytometry, and molecular clonality studies, has decreased the probability of misdiagnosis. Until reliable prognostic markers are available, patients with KFD should have continued long-term follow-up care due to their increased risk of SLE.
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17
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Vu D, Reddy S, Day L, Aydin N, Misra S. Kikuchi-Fujimoto Disease: Unusual Presentation of Rare Disease. Clin Pract 2015; 6:828. [PMID: 27162600 PMCID: PMC4844816 DOI: 10.4081/cp.2016.828] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/27/2015] [Revised: 03/03/2016] [Accepted: 03/13/2016] [Indexed: 11/23/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare, benign disorder that typically follows a self-limiting natural course and was initially described in young females of Asian descent. Its clinical presentation may mimic lymphoproliferative disorders, connective tissue disorders, and chronic infections. This often leads to misdiagnosis and inappropriate treatment. The exact cause of this condition remains unknown although autoimmune processes and certain infectious agents have been associated with the disease. The diagnosis of KFD is made histopathologically. Treatment is supportive and long-term follow-up is recommended due to increased risk of future development of systemic lupus erythematosus. Here we are presenting a case of a patient with an unusual presentation of KFD.
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Affiliation(s)
- Duy Vu
- Texas Tech University Health Sciences Center School of Medicine , Amarillo, TX, USA
| | | | - Lynn Day
- Texas Tech University Health Sciences Center School of Medicine , Amarillo, TX, USA
| | - Nail Aydin
- Texas Tech University Health Sciences Center School of Medicine , Amarillo, TX, USA
| | - Subhasis Misra
- Texas Tech University Health Sciences Center School of Medicine , Amarillo, TX, USA
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