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1
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Erdmann SAB, Do Nguyen D, Wei S. Kikuchi-Fujimoto disease: a differential for fevers and lymphadenopathy. BMJ Case Rep 2025; 18:e263960. [PMID: 40250858 DOI: 10.1136/bcr-2024-263960] [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] [Indexed: 04/20/2025] Open
Abstract
A woman in her early 20s with no significant medical history presented with tender cervical lymphadenopathy, swinging feverish episodes up to 39.6°C, a widespread maculopapular rash and polyarthralgia. She was extensively investigated as an inpatient with multiple imaging studies and blood tests to encompass the potential diagnosis of infection, malignancy or autoimmune disorders. She was discharged after undergoing an excisional cervical lymph node biopsy, which was later reported as consistent with the diagnosis of Kikuchi-Fujimoto disease.
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Affiliation(s)
| | - Daniel Do Nguyen
- Department of Anatomical Pathology, Queen Elizabeth II Medical Centre, PathWest Laboratory Medical WA, Nedlands, Australia
| | - Simon Wei
- General Medicine, Sir Charles Gairdner Hospital, Nedlands, Western Australia, Australia
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2
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Soto-Moreno A, Vílchez-Márquez F, Narváez-Simón M, Castro-Martín J, Ramos-Pleguezuelos FM, Soto-Díaz A, Tercedor-Sánchez J, Arias-Santiago S. Pediatric Kikuchi-Fujimoto Disease: Case Report and Review of Cutaneous and Histopathologic Features in Childhood. Dermatopathology (Basel) 2025; 12:7. [PMID: 39982355 PMCID: PMC11860556 DOI: 10.3390/dermatopathology12010007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2024] [Revised: 01/27/2025] [Accepted: 02/05/2025] [Indexed: 02/22/2025] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare condition characterized by necrotizing lymphadenitis and fever, often associated with immune dysregulation. Histologically, it features necrotic foci with abundant histiocytes and plasmacytoid dendritic cells but notably lacks neutrophils and eosinophils. Recent evidence reveals a notable prevalence among pediatric patients, who may exhibit distinct features compared to adults. We reported the case of an 11-year-old girl presenting with persistent fever, cervical adenopathy, and a malar rash, leading to a diagnosis of KFD following lymph node biopsy, which revealed non-suppurative necrosis and histiocytic infiltration. Empirical treatment with antivirals and antibiotics was ineffective, but corticosteroid therapy achieved symptom remission. A literature review identified 48 relevant studies involving 386 pediatric cases, with histopathological findings consistent with classical descriptions of KFD. Cutaneous involvement was reported in 11.14% of cases, ranging from maculopapular rashes to lupus-like eruptions. Notable complications included the development of systemic lupus erythematous, Sjögren syndrome, and rare instances of hemophagocytic syndrome or central nervous system involvement. Kikuchi-Fujimoto disease should be considered in the differential diagnosis of pediatric patients presenting with fever and lymphadenopathy, taking into account a higher frequency of cutaneous manifestations in pediatric cases. A skin biopsy may be helpful in diagnosing KFD and provide valuable information regarding the potential risk of developing systemic lupus erythematosus in the future.
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Affiliation(s)
- Alberto Soto-Moreno
- Dermatology Department, Hospital Universitario Virgen de Las Nieves, 18012 Granada, Spain; (A.S.-M.); (F.V.-M.); (J.C.-M.); (A.S.-D.); (J.T.-S.)
| | - Francisco Vílchez-Márquez
- Dermatology Department, Hospital Universitario Virgen de Las Nieves, 18012 Granada, Spain; (A.S.-M.); (F.V.-M.); (J.C.-M.); (A.S.-D.); (J.T.-S.)
| | - María Narváez-Simón
- Pathology Derpartment, Hospital Universitario Virgen de Las Nieves, 18012 Granada, Spain (F.M.R.-P.)
| | - Julia Castro-Martín
- Dermatology Department, Hospital Universitario Virgen de Las Nieves, 18012 Granada, Spain; (A.S.-M.); (F.V.-M.); (J.C.-M.); (A.S.-D.); (J.T.-S.)
| | | | - Agustín Soto-Díaz
- Dermatology Department, Hospital Universitario Virgen de Las Nieves, 18012 Granada, Spain; (A.S.-M.); (F.V.-M.); (J.C.-M.); (A.S.-D.); (J.T.-S.)
| | - Jesús Tercedor-Sánchez
- Dermatology Department, Hospital Universitario Virgen de Las Nieves, 18012 Granada, Spain; (A.S.-M.); (F.V.-M.); (J.C.-M.); (A.S.-D.); (J.T.-S.)
| | - Salvador Arias-Santiago
- Dermatology Department, Hospital Universitario Virgen de Las Nieves, 18012 Granada, Spain; (A.S.-M.); (F.V.-M.); (J.C.-M.); (A.S.-D.); (J.T.-S.)
- Granada Institute for Biosanitary Research, 18012 Granada, Spain
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Cadório MJ, Oliveira J, Gama J, Duarte C. Kikuchi-Fujimoto disease and primary Sjögren's syndrome coexisting: A case-based literature review. Mod Rheumatol Case Rep 2025; 9:110-116. [PMID: 39305128 DOI: 10.1093/mrcr/rxae058] [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: 07/09/2024] [Revised: 08/24/2024] [Accepted: 09/17/2024] [Indexed: 01/18/2025]
Abstract
Kikuchi-Fujimoto Disease (KFD), also known as Kikuchi histiocytic necrotising lymphadenitis, is an extremely rare and benign condition that mostly affects young women. It is characterised by lymph node involvement with a predilection for the cervical region, commonly presenting with tender lymphadenopathy and a low-grade fever. The diagnosis requires excisional lymph node biopsy with immunohistochemical analysis. KFD is mostly self-limiting within a few weeks to months, with only some patients requiring symptomatic relief with non-steroidal anti-inflammatory drugs or corticosteroids, and a minority developing recurrent episodes of the disease. Importantly, it has been reported in association with systemic lupus erythematosus and, to a lesser extent, other immune-mediated inflammatory rheumatic diseases, such as Sjögren's syndrome, whose clinical presentation itself may include lymphadenopathy. In this paper, we present an unusual case of a woman with primary Sjögren syndrome (pSS) and a past medical history relevant for lymphoma, sarcoidosis, and thymoma, who later developed KFD, a particularly challenging diagnosis in this setting. We then performed a literature review of the association between KFD and pSS, gathering a total of 13 patients, and focusing epidemiological, clinical, and laboratory data.
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Affiliation(s)
| | - João Oliveira
- Rheumatology Department, Coimbra Local Health Unit, Coimbra, Portugal
| | - João Gama
- Anatomical Pathology Department, Coimbra Local Health Unit, Coimbra, Portugal
| | - Cátia Duarte
- Rheumatology Department, Coimbra Local Health Unit, Coimbra, Portugal
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Cheng R, Lin F, Lu M. Retrospective Analysis of Aseptic Meningitis in Kikuchi-Fujimoto Disease. J Inflamm Res 2024; 17:9319-9324. [PMID: 39588144 PMCID: PMC11587799 DOI: 10.2147/jir.s480056] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/19/2024] [Accepted: 10/31/2024] [Indexed: 11/27/2024] Open
Abstract
Introduction and Objectives Kikuchi-Fujimoto disease (KFD) is self-limiting, has an unknown origin, and predominantly affects the lymph nodes. KFD with aseptic meningitis is rare and diagnostically challenging. This retrospective observational study aimed to elucidate the clinical features and treatment outcomes of KFD, particularly in cases with aseptic meningitis. Methods We conducted this retrospective study to describe KFD to determine the characteristics of the disease, with a particular focus on cases involving aseptic meningitis. Results Our study comprised 103 patients (33 men, 70 women) diagnosed with KFD at Peking University Third Hospital between January 2013 and March 2024. Diagnosis was based on histological examination of lymph node biopsies. The mean age was 25 (range: 16-66) years. Clinical manifestations included fever (100%), cervical pain (79.6%), fatigue (49.5%), headache (44.7%), myalgia (26.2%), and hepatosplenomegaly (23.3%). Biological signs included leukopenia (66.0%) and elevated lactate dehydrogenase (>250 U/L, 83.5%) and ferritin (>300 ng/mL, 44.6%) levels. Forty-three cases improved with nonsteroidal anti-inflammatory drugs (NSAIDs) as monotherapy, whereas 24 required corticosteroid therapy. Four of the 46 patients with headache underwent cerebrospinal fluid analysis, confirming aseptic meningitis. Notably, all four responded well to nonsteroidal anti-inflammatory drugs. Conclusion Our findings highlight the features and outcomes of KFD, particularly its association with aseptic meningitis, which has a favorable prognosis in the absence of corticosteroid therapy.
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Affiliation(s)
- Ran Cheng
- Department of Infectious Diseases, Peking University Third Hospital, Beijing, People’s Republic of China
| | - Fei Lin
- Department of Infectious Diseases, Peking University Third Hospital, Beijing, People’s Republic of China
| | - Ming Lu
- Department of Respiratory and Critical Care Medicine, Peking University Third Hospital, Beijing, People’s Republic of China
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Deb A, Fernandez V, Kilinc E, Bahmad HF, Camps NS, Sriganeshan V, Medina AM. Kikuchi-Fujimoto Disease: A Case Series and Review of the Literature. Diseases 2024; 12:271. [PMID: 39589945 PMCID: PMC11592699 DOI: 10.3390/diseases12110271] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/26/2024] [Revised: 10/22/2024] [Accepted: 10/28/2024] [Indexed: 11/28/2024] Open
Abstract
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, self-limiting disorder characterized by fever typically lasting for 1 week up to 1 month and painful necrotizing lymphadenopathy, primarily affecting young adults of Asian ancestry. Although the exact cause remains unclear, infectious and autoimmune mechanisms have been implicated in the pathogenesis of the disease. In this case series, we aim to describe the histopathological features of KFD over a ten-year period at Mount Sinai Medical Center of Florida, and review the current understanding of its pathogenesis, clinical presentation, diagnosis, and management. A retrospective review of our pathology database between January 2013 and May 2024 was performed to identify patients diagnosed with KFD at our institution. Eight cases of KFD were identified, with a mean age of 35 years (range 24-49) and slight male predilection (5:3). Three patients exhibited leukopenia, and two had concurrent HIV infection. One patient developed systemic lupus erythematosus (SLE), and another developed IgA nephropathy during follow-up. Histopathological examination revealed the characteristic features of KFD, including lymph node architectural effacement, histiocytic infiltration, and necrosis. In conclusion, KFD remains a diagnostic challenge due to its overlapping clinical features with other infectious and autoimmune diseases, particularly SLE. While most cases resolve spontaneously, long-term follow-up is warranted due to the potential for recurrence and autoimmune associations.
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Affiliation(s)
- Arunima Deb
- The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA; (A.D.); (V.F.); (E.K.); (V.S.); (A.M.M.)
| | - Vielka Fernandez
- The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA; (A.D.); (V.F.); (E.K.); (V.S.); (A.M.M.)
| | - Ekim Kilinc
- The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA; (A.D.); (V.F.); (E.K.); (V.S.); (A.M.M.)
| | - Hisham F. Bahmad
- The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA; (A.D.); (V.F.); (E.K.); (V.S.); (A.M.M.)
| | - Nicholas S. Camps
- Department of Internal Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA;
| | - Vathany Sriganeshan
- The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA; (A.D.); (V.F.); (E.K.); (V.S.); (A.M.M.)
- Department of Pathology, Herbert Wertheim College of Medicine, Florida International University, Miami, FL 33199, USA
| | - Ana Maria Medina
- The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA; (A.D.); (V.F.); (E.K.); (V.S.); (A.M.M.)
- Department of Pathology, Herbert Wertheim College of Medicine, Florida International University, Miami, FL 33199, USA
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Sheikh JZ, Kila L, Amin I, Khan U, Casserly B. Kikuchi-Fujimoto Disease With Isolated Mediastinal Lymphadenopathy: A Rare Presentation of an Even Rarer Disease. Cureus 2024; 16:e72921. [PMID: 39628743 PMCID: PMC11613967 DOI: 10.7759/cureus.72921] [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] [Accepted: 11/03/2024] [Indexed: 12/06/2024] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare self-limiting condition presenting as fever and cervical lymphadenopathy, with only two reported cases with isolated mediastinal lymphadenopathy. Lack of awareness about this condition often results in a high rate of misdiagnosis. We present a case of a 29-year-old Indian male with fever, mucocutaneous ulcers, weight loss, and mediastinal lymphadenopathy on CT. Extensive investigations ruled out malignancy and autoimmune and infectious causes. Endobronchial ultrasound-guided fine needle aspiration revealed necrotizing lymphadenitis, indicating KFD, followed by symptomatic management and reassurance. Awareness is crucial to avoid misdiagnosis and unnecessary treatment due to its potentially serious differentials. Long-term follow-up is imperative due to its association with autoimmune conditions.
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Affiliation(s)
| | - Louay Kila
- Respiratory Medicine, University Hospital Limerick, Limerick, IRL
| | - Irfan Amin
- Respiratory Medicine, University Hospital Limerick, Limerick, IRL
| | - Umar Khan
- Respiratory Medicine, University Hospital Limerick, Limerick, IRL
| | - Brian Casserly
- Respiratory Medicine, University Hospital Limerick, Limerick, IRL
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Zheng Y, Jin L, Li X. Differential diagnosis of pediatric cervical lymph node lesions based on simple clinical features. Eur J Pediatr 2024; 183:4929-4938. [PMID: 39285058 DOI: 10.1007/s00431-024-05760-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/14/2024] [Revised: 06/21/2024] [Accepted: 09/01/2024] [Indexed: 10/15/2024]
Abstract
This study aims to to establish a diagnosis model based on simple clinical features for children with cervical histiocytic necrotizing lymphadenitis or malignant lymphoma. Simple clinical features of pediatric patients were analyzed to develop a diagnosis model based on a comparison of classical machine-learning algorithms. This was a single-center retrospective study in a tertiary pediatrics hospital. Pediatric patients treated for cervical histiocytic necrotizing lymphadenitis or malignant lymphoma treated at our institution in recent 5 years were included. Demographic data and laboratory values were recorded and binary logistics regression analysis was applied to select possible predictors to develop diagnostic models with different algorithms. The diagnostic efficiency and stability of each algorithm were evaluated to select the best one to help establish the final model. Eighty-three children were included with 45 cases of histiocytic necrotizing lymphadenitis and 38 cases of malignant lymphoma. Peak temperature, white blood cell count, monocyte percentage, and urea value were selected as possible predictors based on the binary logistics regression analysis, together with imaging features already reported (size, boundary, and distribution of mass). In the ten-round random testing sets, the discriminant analysis algorithm achieved the best performance with an average accuracy of 89.0% (95% CI 86.2-93.6%) and an average AUC value of 0.971 (95% CI 0.957-0.995). CONCLUSION A discriminant analysis model based on simple clinical features can be effective in differential diagnosis of cervical histiocytic necrotizing lymphadenitis and malignant lymphoma in children. Peak body temperature, white blood cell count, and short diameter of the largest mass are significant predictors. WHAT IS KNOWN • Several multivariate diagnostic models for HNL and ML have been proposed based on B-ultrasound or CT features in adults. • The differences between children and adults are nonnegligible in the clinical featues of HNL. WHAT IS NEW • The study firstly report a large-sample diagnostic model between the HNL and MLin pediatric patients. • Non-imaging clinical features has also been proven with quite good diagnostic value.
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Affiliation(s)
- Yangyang Zheng
- Department of Otolaryngology-Head and Neck Surgery, School of Medicine, Shanghai Children's Hospital, Shanghai Jiao Tong University, No. 24, Lane 1400, West Beijing Road, Shanghai, 200040, People's Republic of China
| | - Lei Jin
- Department of Otolaryngology-Head and Neck Surgery, School of Medicine, Shanghai Children's Hospital, Shanghai Jiao Tong University, No. 24, Lane 1400, West Beijing Road, Shanghai, 200040, People's Republic of China
| | - Xiaoyan Li
- Department of Otolaryngology-Head and Neck Surgery, School of Medicine, Shanghai Children's Hospital, Shanghai Jiao Tong University, No. 24, Lane 1400, West Beijing Road, Shanghai, 200040, People's Republic of China.
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8
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Yamashita T, Momose S, Imada H, Takayanagi N, Murakami C, Nagata M, Sawada K, Yamazaki M, Shimizu T, Kikuchi Y, Yamamoto W, Higashi M. The significance of T-BET-positive CD8 T-cells with diminished CD5 expression in Kikuchi-Fujimoto disease. J Clin Exp Hematop 2024; 64:183-190. [PMID: 39085130 PMCID: PMC11528254 DOI: 10.3960/jslrt.24019] [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: 04/15/2024] [Revised: 05/13/2024] [Accepted: 05/14/2024] [Indexed: 08/02/2024] Open
Abstract
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare condition characterized by benign localized lymphadenopathy and clinical symptoms such as fever, sore throat, odynophagia, and leukopenia. Though the etiology of KFD is unknown, this condition is similar to viral infection, including increased infiltration of activated plasmacytoid dendritic cells. KFD exhibits three histological phases that reflect its progression status: proliferative, necrotic, and xanthomatous lesions. The expression loss of pan T-cell markers, such as CD2, CD5, and CD7, of infiltrating T-cells is observed in KFD cases, complicating the distinction from T-cell lymphoma. However, reports on the loss of their expression in KFD have been limited. Furthermore, the precise population of the T-cell subset in KFD is still unclear. Here, we focused on surface markers and transcription factors for T-cell differentiation and analyzed them immunohistochemically in 46 KFD cases. We observed diminished CD5 expression of CD8-positive (CD5dim CD8+) T-cells in the proliferative lesion of KFD cases. Furthermore, these CD5dim CD8+ T-cells expressed T-BET, a master regulator of type 1 helper T-cells. The upregulation of T-BET and downregulation of CD5 in CD8+ T-cells causes dysregulated activation and proliferation of CD8+ T-cells, potentially contributing to the unique histopathological features of KFD. Recognizing the frequent infiltration of T-BET-positive CD5dim CD8+ T-cells in KFD is important for distinguishing it from mature T-cell lymphoma. Our findings suggest that the immune response in KFD shares similarities with viral infections and highlight the importance of characterizing T-BET-positive CD5dim CD8+ T-cell populations for understanding KFD pathogenesis.
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Affiliation(s)
- Takahisa Yamashita
- Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
| | - Shuji Momose
- Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
| | - Hiroki Imada
- Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
| | - Natsuko Takayanagi
- Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
| | - Chiaki Murakami
- Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
| | - Marino Nagata
- Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
| | - Keisuke Sawada
- Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
| | - Mami Yamazaki
- Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
| | - Tomomi Shimizu
- Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
| | - Yukina Kikuchi
- Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
| | - Wataru Yamamoto
- Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
| | - Morihiro Higashi
- Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
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Kottarathil MS, D'Cruze L, Sundaram S, Kalantri SH, Malik A. Spectrum of Kikuchi-Fujimoto Disease: A 10-Year Study in a Tertiary Care Centre in South India. Cureus 2024; 16:e69888. [PMID: 39439650 PMCID: PMC11494157 DOI: 10.7759/cureus.69888] [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] [Accepted: 09/21/2024] [Indexed: 10/25/2024] Open
Abstract
Background Kikuchi-Fujimoto disease (KFD) is a benign lesion of the lymph nodes predominantly seen in younger women and is a condition associated with a good prognosis due to its indolent nature. Since KFD is chiefly a diagnosis of exclusion, it is liable to a higher degree of misdiagnosis. Aim In our study, we attempt to document and analyse the demographic, clinical, and pathological spectrum of cases diagnosed as KFD in a South Indian tertiary care centre over a period of 10 years. Methodology This study was conducted in a retrospective observational manner. Descriptive statistics, including simple frequency and percentage, were used to analyse and summarise the collected data. Results Among the 44 cases obtained, 36 patients were female and eight were males, with an age range of eight to 70 years. The mean age of the affected population was 26 years. Fever was a presenting complaint in 63.6% of cases, and 100% of the cases had lymphadenopathy, chiefly affecting the posterior cervical group of nodes (32/44 cases). Multiple lymph node groups were involved in 22 cases. Most of the nodes were larger than 1 cm in their greatest dimension. On analysis of peripheral smears, 43.2% of patients showed leukopenia. Anti-nuclear antibody (ANA) analysis showed a positive status among two patients, simultaneously establishing the diagnosis of systemic lupus erythematosus (SLE). A history of treated tuberculosis was present in four patients, and one patient had a history of non-compliance with TB treatment. Two patients had recently been diagnosed with TB and were on treatment. The involved nodes were subjected to fine-needle aspiration studies in 13 patients; 12/13 cases showed reactive lymphadenitis, while one case had atypical lymphoid cells. The involved nodes of all the patients were excised and sent for histopathological analysis. The diagnosis of KFD was confirmed on the biopsy. Conclusion In this study, we note the clinical presentations of all the patients diagnosed with KFD and elaborate on the investigations that were employed to verify the diagnosis via exclusion that enabled differentiation of KFD from its close mimickers, which have specific treatment. Keeping this entity in mind is essential in order to prevent overdiagnosis, as most often this condition resolves spontaneously, and in occasional cases, symptomatic treatment is sufficient for its management.
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Affiliation(s)
- Meryl S Kottarathil
- Pathology, Sri Ramachandra Institute of Higher Education and Research, Chennai, IND
| | - Lawrence D'Cruze
- Pathology, Sri Ramachandra Institute of Higher Education and Research, Chennai, IND
| | - Sandhya Sundaram
- Pathology, Sri Ramachandra Institute of Higher Education and Research, Chennai, IND
| | - Suman H Kalantri
- Pathology and Laboratory Medicine, Sankara Nethralaya Hospitals, Chennai, IND
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Kularathna MDSV, Medagama A, Bandara R, Yasarathna D, Dilani MKI, Anuththara T. Kikuchi disease complicated with aseptic meningitis following COVID-19 Vaccination: a case report. J Med Case Rep 2024; 18:270. [PMID: 38840233 PMCID: PMC11155001 DOI: 10.1186/s13256-024-04541-z] [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: 03/13/2022] [Accepted: 04/10/2024] [Indexed: 06/07/2024] Open
Abstract
BACKGROUND Kikuchi Fujimoto disease is a rare self-limiting disorder mainly affecting young Asian females. The typical presentation is unexplained fever with associated cervical lymphadenopathy. It can mimic many sinister diseases such as lymphoma, tuberculosis, and systemic lupus erythematosus. Aseptic meningitis due to Kikuchi disease is extremely rare, and majority were reported from Japan. There have been no published cases of aseptic meningitis due to Kikuchi disease in Sri Lanka. CASE PRESENTATION A 29 years old Sri Lankan female presented with a prolonged fever for three weeks with an associated headache for five days duration. She developed painful cervical lymphadenopathy during the hospital stay. She has been previously well and had been vaccinated against COVID-19 six weeks before. Her lumbar puncture showed lymphocytic pleocytosis with marginally elevated protein levels and reduced ratio of serum to CSF sugar. Lymph node biopsy was consistent with necrotizing lymphadenitis. She was subsequently diagnosed with Kikuchi disease complicated with aseptic meningitis. She responded to corticosteroids well and had an uneventful recovery. CONCLUSION Kikuchi disease is a rare self-limiting disorder that can be complicated with aseptic meningitis on infrequent occasions. Other conditions such as tuberculosis, lymphoma, systemic lupus erythematosus, and adult-onset Still's disease should be considered as differential diagnoses. Knowledge of Kikuchi disease and its complications will prevent unnecessary investigations which delay the early diagnosis and treatment.
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Yu RB, Chen YJ, Chang CH, Chen YL, Chen JW. Kikuchi-Fujimoto Disease Associated With Mycoplasma Pneumoniae Infection. EAR, NOSE & THROAT JOURNAL 2024; 103:NP223-NP225. [PMID: 34605286 DOI: 10.1177/01455613211044225] [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] [Indexed: 11/15/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a self-limited disease that is more common in young Asian women. Typical presentations included fever and cervical lymphadenopathy. The etiology of KFD is unknown, and diagnosis is based mainly on lymph node biopsy. KFD has been reported to be associated with Mycoplasma pneumoniae infection. However, the role of antibiotic treatment is unclear. We reported 2 cases of KFD associated with Mycoplasma pneumoniae infection and were successfully treated with a macrolide.
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Affiliation(s)
- Rui-Bin Yu
- Department of Otolaryngology-Head and Neck Surgery, Catholic Cardinal Tien Hospital, New Taipei City, Taiwan ROC
- Department of Otolaryngology-Head and Neck Surgery, National Taiwan University Hospital, Taipei, Taiwan ROC
| | - Yi-Jia Chen
- Department of Otolaryngology-Head and Neck Surgery, Catholic Cardinal Tien Hospital, New Taipei City, Taiwan ROC
| | - Chun-Hsiang Chang
- Department of Otolaryngology-Head and Neck Surgery, Catholic Cardinal Tien Hospital, New Taipei City, Taiwan ROC
- Department of Otolaryngology-Head and Neck Surgery, National Taiwan University Hospital, Taipei, Taiwan ROC
- School of Medicine, Fu-Jen Catholic University, New Taipei City, Taiwan ROC
| | - Yen-Lin Chen
- School of Medicine, Fu-Jen Catholic University, New Taipei City, Taiwan ROC
- Department of Pathology, Catholic Cardinal Tien Hospital, New Taipei City, Taiwan ROC
| | - Jeng-Wen Chen
- Department of Otolaryngology-Head and Neck Surgery, Catholic Cardinal Tien Hospital, New Taipei City, Taiwan ROC
- Department of Otolaryngology-Head and Neck Surgery, National Taiwan University Hospital, Taipei, Taiwan ROC
- School of Medicine, Fu-Jen Catholic University, New Taipei City, Taiwan ROC
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12
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Lou D, Song Y. Clinical features of histiocytic necrotizing lymphadenitis in children. Eur J Pediatr 2024; 183:1333-1339. [PMID: 38141136 DOI: 10.1007/s00431-023-05391-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/11/2023] [Revised: 12/11/2023] [Accepted: 12/14/2023] [Indexed: 12/24/2023]
Abstract
Due to its nonspecific clinical characteristics, histiocytic necrotizing lymphadenitis (HNL) is often misdiagnosed as a suppurative cervical lymphadenitis and lymphoma. Thus, this study aimed to investigate the clinical characteristics of HNL in pediatric patients. We retrospectively identified 61 patients with histopathologically confirmed HNL. Clinical and laboratory data, including age, sex, clinical manifestations, laboratory investigations, histological discoveries, treatment, and outcomes, were collected from the medical records to determine associations with extracervical lymph node (LN) involvement. The mean age of patients was 9.7 ± 2.8 years (range, 1.5-14.0 years), and the male-to-female ratio was 2.2:1. The most common systemic symptom was fever in all patients. The median pre-admission and total durations of fever were 13.0 (interquartile range [IQR]: 9.0-22.5 days) and 22.0 days (IQR: 17.0-33.0 days), respectively. Patients with temporary fever (< 2 weeks) had a higher peak temperature and were more likely to undergo LN biopsy after admission than those with a prolonged fever (≥ 2 weeks). Multivariate analysis revealed that peak temperature ≥ 40 °C was significantly associated with a longer fever duration (P = 0.023). Laboratory values showed leukopenia (68.9%), which presented more frequently in solitary cervical LNs than in extracervical LNs (82.4% vs. 52.9%, p = 0.027) in patients with prolonged fever. CONCLUSIONS HNL is often misdiagnosed in older children with persistent fever and lymphadenopathy, leading to unnecessary diagnostic tests and evaluations, inappropriate antibiotic administration, and mismanagement. A multidisciplinary team, including primary care providers, rheumatologists, and pathologists, can improve patient outcomes by increasing their awareness of this rare condition. WHAT IS KNOWN • Histiocytic necrotizing lymphadenitis (HNL) is characterized by fever, leukopenia, and neck lymphadenopathy with unknown etiology. • The lack of neutrophils or eosinophils in the histology, immunohistochemistry results help distinguish HNL from infectious causes. Although HNL is a self-limiting disease, antibiotics and steroid treatments were used inappropriately. WHAT IS NEW • A fever peak ≥ 40 °C was associated with a longer fever duration in HNL patients. Leukopenia presented more frequently in solitary cervical lymph node (LNs) than in extracervical LNs inpatients with prolonged fever. • Steroids are not recommended as a routine treatment, however, in some severe or relapsing cases with persistent symptoms, prednisolone (5 mg twice a day for 2 days) or other steroids (an equivalent dose of prednisolone) responded favorably.
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Affiliation(s)
- DanDan Lou
- Department of Pediatrics, The Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, 710004, People's Republic of China
| | - Ye Song
- Department of Pediatrics, The First Affiliated Hospital of Air Force Medical University, No. 127 Changle West Road, Xincheng District, Xi'an, Shaanxi, 710032, People's Republic of China.
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13
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Mariani RA, Courville EL. Reactive Lymphadenopathy in the Pediatric Population with a Focus on Potential Mimics of Lymphoma. Semin Diagn Pathol 2023; 40:371-378. [PMID: 37295994 DOI: 10.1053/j.semdp.2023.05.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/08/2023] [Accepted: 05/30/2023] [Indexed: 06/12/2023]
Abstract
Benign lymphadenopathy is common in the pediatric population and may be clinically striking. As in adults, lymph node evaluation in pediatric patients requires careful morphologic and immunohistochemical assessment and clinical contextualization of the findings. It is important for the pathologist to be familiar with benign and reactive conditions that may mimic malignancy. This review presents non-neoplastic or indolent processes or patterns of lymphoid hyperplasia that may be confused with or raise the differential of lymphoma, with a focus on those more commonly encountered in the pediatric/adolescent population.
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Affiliation(s)
- Rachel A Mariani
- University of Arizona College of Medicine-Phoenix, Dept. of Pathology & Laboratory Medicine, USA
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14
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Lee DE, Pak MG, Kim SH, Chandler C. Kikuchi-Fujimoto Disease: A Case of Febrile Cervical Lymphadenopathy With Hematological Abnormalities. Cureus 2023; 15:e47533. [PMID: 37877108 PMCID: PMC10593182 DOI: 10.7759/cureus.47533] [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] [Accepted: 10/23/2023] [Indexed: 10/26/2023] Open
Abstract
Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a benign but rare disorder associated with febrile cervical lymphadenopathy in young adults. Here, we discuss a case of a young female patient presenting with left tender cervical lymphadenopathy that progressed bilaterally with a fever of unknown origin. Laboratory parameters showed persistent leukopenia, especially neutropenia, which fluctuated with the degree of symptom severity. Two months were taken to confirm the diagnosis of KFD based on the histological interpretation of the lymph node biopsy. Supportive management with analgesics and paracetamol formed the main treatment. This case highlights the challenges and importance of diagnosing KFD to exclude other serious conditions such as lymphoma, tuberculosis, or lupus lymphadenitis that share similar clinical manifestations as KFD.
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Affiliation(s)
- Dong Eun Lee
- Department of Acute Medicine, University Hospitals Sussex NHS Foundation Trust, Worthing, GBR
| | - Min Gyoung Pak
- Department of Pathology, Dong-A University College of Medicine, Busan, KOR
| | - Sung-Hyun Kim
- Department of Internal Medicine, Dong-A University College of Medicine, Busan, KOR
| | - Christopher Chandler
- Department of Acute Medicine, University Hospitals Sussex NHS Foundation Trust, Worthing, GBR
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15
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Rana MBM, Fatima M, Rana IM, Haseeb Ul Rasool M, Abosheaishaa H, Abrudescu A, Bandagi S. Kikuchi-Fujimoto Disease With Systemic Lupus Erythematosus and Systemic Sclerosis Overlap: A Unique Clinical Presentation. Cureus 2023; 15:e44986. [PMID: 37822435 PMCID: PMC10562878 DOI: 10.7759/cureus.44986] [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] [Accepted: 09/10/2023] [Indexed: 10/13/2023] Open
Abstract
Kikuchi-Fujimoto Disease (KFD), or histiocytic necrotizing lymphadenitis (HNL), is a rare self-limiting disorder presenting with fever and swollen lymph nodes. It is characterized by the focal proliferation of reticular cells, the presence of nuclear debris, and histiocytes. In advanced cases, it can present with hepato-splenomegaly and generalized lymphadenopathy. Historically, it has been associated with viral infections, as it frequently was found to be associated with upper respiratory symptoms. Alternative explanations include the immune response of T-cells leading to alteration in CD8-positive T-cell-mediated cell apoptosis. It is also speculated that KFD can be associated with rheumatological autoimmune diseases. We present a case of a 21-year-old African American female with a known diagnosis of systemic lupus erythematosus (SLE)-systemic sclerosis (SS) overlap presented with febrile lymphadenopathy and was diagnosed to have HNL on histological exam of lymph node biopsy.
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Affiliation(s)
| | - Minahil Fatima
- Internal Medicine, Services Hospital Lahore, Lahore, PAK
| | - Iqra M Rana
- Internal Medicine, Allama Iqbal Medical College, Lahore, PAK
| | | | - Hazem Abosheaishaa
- Internal Medicine, Icahn School of Medicine at Mount Sinai, Queens Hospital Center, New York City, USA
- Internal Medicine/Gastroenterology, Cairo University, Cairo, EGY
| | - Adriana Abrudescu
- Rheumatology, Icahn School of Medicine at Mount Sinai, Queens Hospital Center, New York City, USA
| | - Sabiha Bandagi
- Rheumatology, Icahn School of Medicine at Mount Sinai, Queens Hospital Center, New York City, USA
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16
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Craig JW, Farinha P, Jiang A, Lytle A, Skinnider B, Slack GW. Kikuchi-Fujimoto Disease Following COVID-19 Vaccination: Experience at a Population-Based Referral Center. Am J Clin Pathol 2023; 160:114-118. [PMID: 37526500 DOI: 10.1093/ajcp/aqad032] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2022] [Accepted: 03/03/2023] [Indexed: 08/02/2023] Open
Abstract
OBJECTIVES Multiple case reports describe Kikuchi-Fujimoto disease (KFD) following COVID-19 vaccination, but the true nature of this phenomenon is unknown. The purpose of this study was to further assess the relationship between KFD and COVID-19 vaccination at the population level. METHODS Confirmed KFD cases from January 2018 to April 2022 were identified from provincial pathology archives and analyzed in the context of vaccination statistics from public health resources. RESULTS Our statistical models provide evidence of a temporal association between KFD and both antecedent COVID-19 vaccine administration as well as age-stratified vaccination rates. Eight new cases of plausible COVID-19 vaccine-associated KFD are presented, collectively exhibiting clinical and pathologic features that overlap substantially with those of idiopathic KFD. CONCLUSIONS Our findings indicate that KFD is observed in association with COVID-19 vaccination and suggest that mechanistic studies are warranted.
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Affiliation(s)
- Jeffrey W Craig
- Department of Pathology, University of Virginia Health System, Charlottesville, VA, USA
| | - Pedro Farinha
- Centre for Lymphoid Cancer and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada
| | - Aixiang Jiang
- Centre for Lymphoid Cancer and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada
| | - Andrew Lytle
- Centre for Lymphoid Cancer and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, BC Cancer, Vancouver, Canada
| | - Brian Skinnider
- Centre for Lymphoid Cancer and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada
| | - Graham W Slack
- Centre for Lymphoid Cancer and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada
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17
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Liu J, Zheng Q, Shi L, Zhao Z, Chen X, Wang T, Chen X. A retrospective study of 134 patients with cervical region Kikuchi-Fujimoto disease. Laryngoscope Investig Otolaryngol 2023; 8:865-869. [PMID: 37621285 PMCID: PMC10446312 DOI: 10.1002/lio2.1086] [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: 11/23/2022] [Revised: 05/04/2023] [Accepted: 05/23/2023] [Indexed: 08/26/2023] Open
Abstract
Background To explore the clinical and laboratory features, therapy and prognosis of Kikuchi-Fujimoto disease (KFD) in the cervical region. Methods We retrospectively reviewed the medical records of 134 patients who were diagnosed and treated with KFD from January 2000 to May 2022 in Fujian Medical University Union Hospital (Fujian, China). Their clinical characteristics, affected lymph node size, imaging examinations, and laboratory study results were analyzed. Results The mean patient age was 24.9 years, and the male-female ratio was 1:1.73. Fever (55.2%, n = 74) was the most common clinical manifestation. Leukopenia (49.3%) was the commonest reported laboratory abnormality. A majority (65.7%) of the 134 patients presented with bilateral nodal involvement. Conclusion KFD should be considered as a possible diagnosis in a female patient under the age of 30 presenting with cervical lymphadenopathy, fever, leukopenia, and elevated LDH. Level of Evidence 4.
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Affiliation(s)
- Jianzhi Liu
- Department of OtolaryngologyFujian Medical University Union HospitalFuzhouFujianChina
| | - Qiaoling Zheng
- Department of PathologyFujian Medical University Union HospitalFuzhouFujianChina
| | - Liangwen Shi
- Department of OtolaryngologyFujian Medical University Union HospitalFuzhouFujianChina
| | - Zhiwei Zhao
- Department of OtolaryngologyZhangzhou Affiliated Hospital of Fujian Medical UniversityZhangzhouFujianChina
| | - Xuxiang Chen
- Department of OtolaryngologyFujian Medical University Union HospitalFuzhouFujianChina
| | - Taiqin Wang
- Department of OtolaryngologyFujian Medical University Union HospitalFuzhouFujianChina
| | - Xiaoqiang Chen
- Department of OtolaryngologyFujian Medical University Union HospitalFuzhouFujianChina
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18
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Hartmann S, Melle F, Motta G, Agostinelli C, Sabattini E, Pileri S, Hansmann ML. Clonal T-cell proliferations occasionally occur in Kikuchi-Fujimoto disease. Hum Pathol 2023; 138:103-111. [PMID: 37331528 DOI: 10.1016/j.humpath.2023.06.003] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/13/2023] [Accepted: 06/13/2023] [Indexed: 06/20/2023]
Abstract
Kikuchi-Fujimoto disease (KFD) is a benign self-limiting disorder that frequently leads to swelling of cervical lymph nodes in young women. It has a characteristic histologic appearance with sharply demarcated foci containing apoptotic debris, histiocytes, and proliferating large T-cells. Since in the past years, core needle biopsies have been increasingly used for diagnostic work-up, a small biopsy of the pathognomonic proliferating T-cell foci may lead to misinterpretation as a large T-cell neoplasia. The aim of the present study therefore was to analyze how frequently clonal T-cell receptor (TCR) amplificates may be obtained in KFD using a commonly used TCR gamma rearrangement clonality assay. In 88 KFD cases, TCR gamma clonality assays could be successfully applied. Clonal peaks of TCR gamma in front of a polyclonal background were observed in 15 cases (18%). The investigated clinical parameters (age, gender, extent of infiltration of the lymph node, percentage of proliferative compartment) did not differ between patients with detectable TCR gamma clones from those patients who had polyclonal TCR gamma results. Our study therefore demonstrates that clonal TCR gamma amplificates may be obtained in any type of KFD and that an over-interpretation of clonal T-cell proliferates in diagnostically equivocal material should be avoided.
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Affiliation(s)
- Sylvia Hartmann
- Dr. Senckenberg Institute of Pathology, Goethe University Frankfurt Am Main, D-60590 Frankfurt am Main, Germany.
| | - Federica Melle
- Division of Haematopathology, Haematology Programme, IEO European Institute of Oncology IRCCS, 20121 Milan, Italy
| | - Giovanna Motta
- Division of Haematopathology, Haematology Programme, IEO European Institute of Oncology IRCCS, 20121 Milan, Italy
| | - Claudio Agostinelli
- Haematopathology Unit, IRCCS Azienda Ospedaliero-Universitaria of Bologna, 40138 Bologna, Italy; Department of Medical and Surgical Sciences, University of Bologna, 40138 Bologna, Italy
| | - Elena Sabattini
- Haematopathology Unit, IRCCS Azienda Ospedaliero-Universitaria of Bologna, 40138 Bologna, Italy
| | - Stefano Pileri
- Division of Haematopathology, Haematology Programme, IEO European Institute of Oncology IRCCS, 20121 Milan, Italy
| | - Martin-Leo Hansmann
- Frankfurt Institute for Advanced Studies, 60438 Frankfurt am Main, Germany; Institute of General Pharmacology and Toxicology, Goethe University Frankfurt am Main, D-60590 Frankfurt am Main, Germany
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19
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Arya P V A, Alam MM, Bernhisel A, Degirolamo A, Huang R. Diagnostic Dilemma in a Case of Necrotizing Lymphadenitis With Macrophage Activation Syndrome. Cureus 2023; 15:e42267. [PMID: 37605695 PMCID: PMC10440125 DOI: 10.7759/cureus.42267] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/21/2023] [Indexed: 08/23/2023] Open
Abstract
Necrotizing lymphadenitis is a histological diagnosis that can arise from various conditions, including lupus lymphadenitis (LL), Kikuchi disease (KD), and infectious causes. Distinguishing between Kikuchi disease and lupus lymphadenitis can be challenging in clinical practice. In this report, we present the clinical scenario of a young female patient with lymphadenopathy and elucidate the process through which we ultimately arrived at a diagnosis of systemic lupus erythematosus (SLE) with macrophage activation syndrome. This case underscores the significance of recognizing Kikuchi disease as a condition that can mimic lupus and sheds light on the distinguishing features of necrotizing lymphadenitis, with a particular focus on Kikuchi disease and lupus lymphadenitis.
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Affiliation(s)
| | - Md Mashiul Alam
- Cardiovascular Disease, Mayo Clinic, Rochester, USA
- Internal Medicine, Bridgeport Hospital, Bridgeport, USA
| | - Andrew Bernhisel
- Pathology, Yale School of Medicine, Yale University, New Haven, USA
| | | | - Rex Huang
- Rheumatology, Yale University, New Haven, USA
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20
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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: 24] [Impact Index Per Article: 12.0] [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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21
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Betancur V, Net J, Chapman J, Yepes M. Kikuchi-Fujimoto-like lymphadenopathy following COVID-19 vaccine: diagnosis and management. BMJ Case Rep 2022; 15:15/12/e252030. [PMID: 36585044 PMCID: PMC9809255 DOI: 10.1136/bcr-2022-252030] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022] Open
Abstract
A woman in her mid 40s presented for breast imaging after 1 week of painful and enlarged right axillary lymphadenopathy. She denied history of fever, weight loss, night sweats fatigue, cat scratch or other trauma. She received the second dose of Pfizer COVID-19 vaccine 3 months previously on the contralateral arm. A mammogram demonstrated a single, asymmetric, large and dense right axillary lymph node. Ultrasound confirmed a 2.5 cm lymph node with cortical thickening of 0.6 cm. Ultrasound-guided core biopsy showed necrotising lymphadenitis with associated aggregates of histiocytes and plasmacytoid dendritic cells. Potential causes of necrotising adenitis including Bartonella, tuberculosis, Epstein-Barr Virus, herpes simplex virus, systemic lupus erythematosus and lymphoma were excluded. In the absence of any identifiable infectious or autoimmune causes, and given the temporal relatedness with vaccine administration, it was determined that the Kikuchi-Fujimoto-like necrotising lymphadenitis was likely secondary to the COVID-19 vaccine. To date, there has been no casual association made between the COVID-19 vaccine and KFD necrotising lymphadenitis.
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Affiliation(s)
- Veronica Betancur
- Department of Radiology, University of Miami Miller School of Medicine, Miami, Florida, USA
| | - Jose Net
- Department of Radiology, University of Miami Miller School of Medicine, Miami, Florida, USA
| | - Jennifer Chapman
- Department of Pathology, University of Miami School of Medicine, Miami, Florida, USA
| | - Monica Yepes
- Department of Radiology, University of Miami Miller School of Medicine, Miami, Florida, USA
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22
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Chisholm KM, Bohling SD, Tsuchiya KD, Paulson VA. A Malignant Mimicker: Features of Kikuchi-Fujimoto Disease in the Pediatric Population. Pediatr Dev Pathol 2022; 25:538-547. [PMID: 35583198 DOI: 10.1177/10935266221103882] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
BACKGROUND Kikuchi-Fujimoto disease (KFD) is a rare, benign, and self-limited disease that presents with cervical lymphadenopathy and systemic symptoms. Histologic evaluation is often necessary to differentiate KFD from other entities. METHODS Electronic medical records and diagnostic material were reviewed for 14 children diagnosed with KFD and 6 children diagnosed with infectious mononucleosis (IM) from 2013-2021. Four cases of KFD were further characterized using targeted DNA-based next-generation sequencing. RESULTS Systemic symptoms were present in 86% (n = 12/14) of KFD patients, the most common being fever. Laboratory values worrisome for malignancy included cytopenia(s) (n = 9/12), elevated ESR and/or CRP (n = 9/12), elevated ferritin (n = 7/7), and elevated LDH (n = 7/10). Histologically, lymph nodes showed characteristic necrotic foci without neutrophils surrounded by MPO+ "crescentic" histiocytes. Immunoblasts and CD123+ plasmacytoid dendritic cells (pDCs) were also increased surrounding the necrosis. IM lymph nodes showed similar features when necrosis was present but increases in pDCs were patchy and rare neutrophils were seen in the necrotic foci. Molecular analysis of 4 KFD cases did not identify pathogenic variants. CONCLUSION While the signs/symptoms of KFD are worrisome, there are pathologic features that help differentiate it from potential mimics. We did not identify characteristic molecular features to aid in the work-up of these cases.
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Affiliation(s)
- Karen M Chisholm
- Department of Laboratories, 7274Seattle Children's Hospital, Seattle, WA, USA.,Department of Laboratory Medicine and Pathology, 7284University of Washington, Seattle, WA, USA
| | - Sandra D Bohling
- Department of Laboratories, 7274Seattle Children's Hospital, Seattle, WA, USA.,Department of Laboratory Medicine and Pathology, 7284University of Washington, Seattle, WA, USA
| | - Karen D Tsuchiya
- Department of Laboratory Medicine and Pathology, 7284University of Washington, Seattle, WA, USA
| | - Vera A Paulson
- Department of Laboratory Medicine and Pathology, 7284University of Washington, Seattle, WA, USA
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23
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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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24
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Naoi Y, Tachibana T, Wani Y, Hotta M, Haruna K, Komatsubara Y, Kuroda K, Fushimi S, Nagatani T, Kataoka Y, Nishizaki K, Sato Y, Ando M. The fine-needle aspiration cytology and clinical findings of Kikuchi-Fujimoto disease in pediatric patients: a retrospective clinical study. Acta Otolaryngol 2022; 142:340-344. [PMID: 35235442 DOI: 10.1080/00016489.2022.2040744] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/01/2022]
Abstract
BACKGROUND Histological evaluation of lymph node is crucial for the definitive diagnosis of Kikuchi-Fujimoto disease (KFD). However, lymph node biopsy under local anesthesia is often difficult in pediatric patients. OBJECTIVES We evaluated cytological findings for pediatric patients with prolonged cervical lymphadenitis clinically suggestive of KFD and investigated the clinical characteristics of patients diagnosed with KFD by fine-needle aspiration cytology (FNAC). METHODS This retrospective clinical study included 58 Japanese pediatric patients with cervical lymphadenitis who underwent FNAC. RESULTS Cytological diagnosis was KFD for 22 and suspicion of KFD for 11 patients. The remaining 25 patients were diagnosed with non-specific lymphadenitis (NSL). Tenderness was independently associated with a higher frequency of both KFD in narrow and broad senses, compared with NSL (p = .009; p = .038). The percentage of patients who underwent FNAC within 28 days from symptom onset tended to be higher among patients with KFD in a narrow sense than those with NSL (p = .052). CONCLUSION This study indicated that the period from symptom onset to FNAC (<28 days) and the symptom of tenderness were associated with the cytological diagnosis of KFD.
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Affiliation(s)
- Yuto Naoi
- Department of Otolaryngology Head and Neck Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan
| | - Tomoyasu Tachibana
- Department of Otolaryngology, Japanese Red Cross Society Himeji Hospital, Himeji City, Japan
| | - Yoji Wani
- Department of Pathology, Japanese Red Cross Society Himeji Hospital, Himeji City, Japan
| | - Machiko Hotta
- Department of Pathology, Japanese Red Cross Society Himeji Hospital, Himeji City, Japan
| | - Katsuya Haruna
- Department of Inspection Technology, Japanese Red Cross Society Himeji Hospital, Himeji City, Japan
| | - Yasutoshi Komatsubara
- Department of Otolaryngology, Japanese Red Cross Society Himeji Hospital, Himeji City, Japan
| | - Kazunori Kuroda
- Department of Otolaryngology, Japanese Red Cross Society Himeji Hospital, Himeji City, Japan
| | - Soichiro Fushimi
- Department of Pathology, Japanese Red Cross Society Himeji Hospital, Himeji City, Japan
| | - Tami Nagatani
- Department of Inspection Technology, Japanese Red Cross Society Himeji Hospital, Himeji City, Japan
| | - Yuko Kataoka
- Department of Otolaryngology Head and Neck Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan
| | - Kazunori Nishizaki
- Department of Otolaryngology Head and Neck Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan
| | - Yasuharu Sato
- Department of Clinical Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan
| | - Mizuo Ando
- Department of Otolaryngology Head and Neck Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama City, Japan
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25
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Ferry JA. Update from the 5th Edition of the World Health Organization Classification of Head and Neck Tumors: Hematolymphoid Proliferations and Neoplasia. Head Neck Pathol 2022; 16:101-109. [PMID: 35312979 PMCID: PMC9018906 DOI: 10.1007/s12105-022-01411-2] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/09/2021] [Accepted: 12/31/2021] [Indexed: 10/18/2022]
Abstract
In the 5th edition of the World Health Organization (WHO) Classification of Head and Neck Tumours, the discussion of hematolymphoid proliferations is substantially reorganized and expanded in comparison to the prior edition. The 5th edition includes, in addition to hematolymphoid neoplasms, reactive lymphoid proliferations. Much more information on hematolymphoid proliferations that commonly affect cervical lymph nodes, in addition to those affecting extranodal sites in the head and neck, is included. For the first time, there are dedicated sections on multiple entities, including recently described lymphoproliferative disorders such as EBV+ mucocutaneous ulcer and pediatric-type follicular lymphoma, and several types of histiocytic neoplasms. Tremendous advances have been made in understanding the genetic features that underlie the pathogenesis of hematolymphoid neoplasms, and these have been incorporated into the WHO Classification.
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Affiliation(s)
- Judith A Ferry
- Harvard Medical School, Massachusetts General Hospital, 55 Fruit Street, Boston, 02114, MA, USA.
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26
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Shahrokh S, Hasan A, Alim S, Hebert M, Rizvi K. Kikuchi-Fujimoto Disease Complicated by Rheumatoid Arthritis, Type 1 Diabetes Mellitus, and Hypothyroidism. Cureus 2022; 14:e21008. [PMID: 35154980 PMCID: PMC8818265 DOI: 10.7759/cureus.21008] [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] [Accepted: 01/06/2022] [Indexed: 11/08/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare, benign, self-limited syndrome characterized by tender lymphadenopathy and low-grade fever. It may also present with rash, arthritis, fatigue, and splenomegaly. Data on the disease is limited, and its etiology remains largely unknown. Here, we present the case of a 30-year-old female with a medical history of rheumatoid arthritis (RA), previously treated with etanercept, type 1 diabetes mellitus (DM-1), and Hashimoto’s hypothyroidism; she was brought in to an emergency department (ED) in Houston after a generalized tonic-clonic seizure and loss of consciousness. She was hypoglycemic, which was thought to have caused her DM-1 and seizure. CT scan of her chest showed multiple enlarged lymph nodes throughout the neck, superior mediastinum, and axilla, along with interstitial edema and bilateral pleural effusions. She was treated with dextrose drip and regained her consciousness. However, she had persistent pancytopenia, low-grade fever, and tender axillary lymphadenopathy. Infectious workup for tuberculosis (TB), human immunodeficiency virus (HIV), herpes simplex virus (HSV), Epstein-Barr virus (EBV), and parvovirus B-19 were negative. Her bone marrow biopsy revealed iron-deficiency anemia, while excisional axillary lymph node biopsy showed extensive necrosis consistent with KFD. She was treated with supportive care. Her neutrophilic fever resolved, and she was discharged home after 48-hours of remaining afebrile. Six months after her hospitalization, the patient remained well, and her complete blood count showed no abnormalities. Due to the non-specific clinical features and laboratory findings of KFD, it is commonly misdiagnosed as infectious, autoimmune, or malignant lymphadenitis, leading to excessive diagnostic tests and unnecessary treatments. Physicians need to be cognizant of KFD and consider it in young patients presenting with tender lymphadenopathy, low-grade fevers, and leukopenia. To our best knowledge, this is the first reported case of a patient with concurrent RA, Hashimoto’s hypothyroidism, and KFD. This report elucidates the autoimmune nature of KFD and its association with other autoimmune diseases.
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Zhang R, Liang L, Li D, Bai Y, Li X. Analysis of the clinical manifestations and 18F-FDG PET-CT findings in 40 patients with histiocytic necrotizing lymphadenitis. Medicine (Baltimore) 2021; 100:e27189. [PMID: 34477179 PMCID: PMC8415950 DOI: 10.1097/md.0000000000027189] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/18/2021] [Accepted: 08/19/2021] [Indexed: 01/05/2023] Open
Abstract
Histiocytic necrotizing lymphadenitis (HNL) is a rare, benign, and self-limiting inflammatory disease that mainly involves the lymph nodes. There is a lack of large sample studies concerning the clinical manifestations and imaging features of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) of HNL.The clinical symptoms, laboratory examination results, 18F-FDG PET/CT imaging features, and treatment outcome were investigated in this retrospective study.A total of 40 HNL patients were recruited. The onset age was between 14 and 65 years, with a median of 25 years. The white blood cell count was 3.9 (2.9, 7.1) × 109/L, C-reactive protein level was 20.2 (6.6, 63.8) mg/L, erythrocyte sedimentation rate was 29.0 (18.0,45.0) mm/h, and ferritin was 616.5 (205.6, 2118.1) ng/mL. An abnormal liver function was observed in 23 patients. 18F-FDG PET-CT showed that an abnormal lymph node metabolism was observed in 38 patients, among which the highest 18F-FDG maximal standard uptake value (SUVmax) of the lymph nodes ranged between 3.4 and 41.9; the nodes were mainly distributed in the neck and axilla regions. Meanwhile, a total of 2502 lymph nodes (721 lymph nodes with a short axis greater than 10 mm) were found in the 38 patients, including 1837 lymph nodes with an 18F-FDG SUVmax ≥ 2.5. The 18F-FDG SUVmax of the spleen ranged from 2.5 to 9.2 in 20 patients, while that of central and peripheral bone marrow ranged from 2.7 to 36.0 in 30 patients. After follow-up for an average period of 1 month, the symptoms improved after prednisone treatment.HNL often occurs in adolescents. Scanning with 18F-FDG PET/CT showed that most patients had multiple involved lymph nodes that were hypermetabolic, and only few lymph nodes are enlarged. Besides, the spleen or central and peripheral bone marrow could sometimes be hypermetabolic. Glucocorticoid treatment for the HNL patients is effective.
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Affiliation(s)
- Rui Zhang
- Department of Respiratory and Critical Care Medicine, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Lidan Liang
- Department of Radiology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Daoming Li
- Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Yuling Bai
- Emergency Intensive Care Unit, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Xiangzhou Li
- Department of Nuclear Medicine, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
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28
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Spiridonov IN, Asaulenko ZP, Krivolapov YA. [Analysis of the phenotypic heterogeneity of CD123-positive cells in Kikuchi-Fujimoto disease using a sequential immunoperoxidase labeling and erasing method]. Arkh Patol 2021; 83:36-44. [PMID: 34278759 DOI: 10.17116/patol20218304136] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare disease that is clinically manifested mainly by fever and lymphadenopathy. KFD was originally believed to occur primarily in East Asia women, this disease was subsequently described in all ethnic groups worldwide. The important differential diagnostic feature of KFD is the detection of CD123-expressing plasmocytoid dendritic cells (PDCs) in the tissue of the affected lymph node. The standard immunohistochemical staining method has sufficient sensitivity and specificity to detect CD123, but it gives no way of judging the possible phenotypic heterogeneity of cells with CD123 expression. OBJECTIVE To identify the phenotypic heterogeneity of CD123-expressing cells in the affected lymph nodes in patients with KFD by a sequential immunoperoxidase labeling and erasing (SIMPLE) method. MATERIAL AND METHODS Excision biopsies of lymph nodes were examined in 3 patients with KFD. After an immunohistochemical reaction using a single antibody, the tissue specimen was digitized with a Pannoramic 250 Flash III scanner (3DHISTECH, Hungary), then the cover glass was removed from the section, the specimen was hydrated and placed in a specialized buffer. Then the following primary antibody was applied to the washed tissue specimen and further immunohistochemical reaction and scanning were performed. As a result, each tissue specimen was sequentially stained in reactions with 4 antibodies. The microphotographs of specimens stained in a reaction with anti-CD123 antibody showed positive cells for their identification in the Pannoramic Viewer program (3DHISTECH, Hungary) on the remaining microphotographs displaying the expression of the other 3 markers. The selected fields of view were exported to a JPG format. RESULTS Assessing the co-expression of the antigens CD123, MNDA, CD68, and TCL1A detected 4 CD123+ cell subpopulations: No. 1. CD68+/ MNDA+/ TCL1A+; No. 2. CD68+/ MNDA+/ TCL1A-; No. 3. CD68+/ MNDA-/ TCL1A+; No. 4. CD68-/ MNDA-/ TCL1A+. CONCLUSION SIMPLE has shown the phenotypic heterogeneity of CD123-positive cells (some of them may be PDCs) and could identify 4 immunophenotypically distinct subpopulations in the affected lymph nodes in patients with KFD. Further investigations are needed to define the role of subpopulations in the pathogenesis of KFD and other diseases.
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Affiliation(s)
- I N Spiridonov
- I.I. Mechnikov North-Western State Medical University of the Ministry of Health of Russia, St. Petersburg, Russia
| | - Z P Asaulenko
- I.I. Mechnikov North-Western State Medical University of the Ministry of Health of Russia, St. Petersburg, Russia.,Saint Petersburg City Hospital Forty, St. Petersburg, Russia
| | - Yu A Krivolapov
- I.I. Mechnikov North-Western State Medical University of the Ministry of Health of Russia, St. Petersburg, Russia
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29
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Yu SC, Chang KC, Wang H, Li MF, Yang TL, Chen CN, Chen CJ, Chen KC, Shen CY, Kuo PY, Lin LW, Lin YM, Lin WC. Distinguishing lupus lymphadenitis from Kikuchi disease based on clinicopathological features and C4d immunohistochemistry. Rheumatology (Oxford) 2021; 60:1543-1552. [PMID: 33169134 DOI: 10.1093/rheumatology/keaa524] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2020] [Revised: 06/18/2020] [Accepted: 07/07/2020] [Indexed: 01/20/2023] Open
Abstract
OBJECTIVES Distinguishing Kikuchi disease (KD) from lupus lymphadenitis (LL) histologically is nearly impossible. We applied C4d immunohistochemical (IHC) stain to develop diagnostic tools. METHODS We retrospectively investigated clinicopathological features and C4d IHC staining in an LL-enriched development cohort (19 LL and 81 KD specimens), proposed risk stratification criteria and trained machine learning models, and validated them in an external cohort (2 LL and 55 KD specimens). RESULTS Clinically, we observed that LL was associated with an older average age (33 vs 25 years; P=0.005), higher proportion of biopsy sites other than the neck [4/19 (21%) vs 1/81 (1%); P=0.004], and higher proportion of generalized lymphadenopathy compared with KD [9/16 (56%) vs 7/31 (23%); P=0.028]. Histologically, LL involved a larger tissue area than KD did (P=0.006). LL specimens exhibited more frequent interfollicular pattern [5/19 (26%) vs 3/81 (4%); P=0.001] and plasma cell infiltrates (P=0.002), and less frequent histiocytic infiltrates in the necrotic area (P=0.030). Xanthomatous infiltrates were noted in 6/19 (32%) LL specimens. Immunohistochemically, C4d endothelial staining in the necrotic area [11/17 (65%) vs 2/62 (3%); P<10-7], and capillaries/venules [5/19 (26%) vs 7/81 (9%); P=0.048] and trabecular/hilar vessels [11/18 (61%) vs 8/81 (10%); P<10-4] in the viable area was more common in LL. During validation, both the risk stratification criteria and machine learning models were superior to conventional histological criteria. CONCLUSIONS Integrating clinicopathological and C4d findings could distinguish LL from KD.
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Affiliation(s)
- Shan-Chi Yu
- Department of Pathology and Graduate Institute of Pathology, College of Medicine, National Taiwan University, Taipei, Taiwan.,Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan.,Graduate Institute of Microbiology, College of Medicine, National Taiwan University, Taipei, Taiwan
| | - Kung-Chao Chang
- Department of Pathology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan
| | - Hsuan Wang
- Department of Pathology, National Taiwan University Hospital Hsin-Chu Branch, Hsinchu, Taiwan
| | - Meng-Fang Li
- Department of Pathology, National Taiwan University Hospital Yun-Lin Branch, Yunlin, Taiwan
| | - Tsung-Lin Yang
- Department of Otolaryngology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
| | - Chun-Nan Chen
- Department of Otolaryngology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
| | - Chih-Jung Chen
- Department of Pathology, Taichung Veterans General Hospital, Taichung, Taiwan.,School of Medicine, Chung Shan Medical University, Taichung, Taiwan
| | - Ko-Chin Chen
- Department of Pathology, Changhua Christian Hospital, Changhua, Taiwan
| | - Chieh-Yu Shen
- Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
| | - Po-Yen Kuo
- Department of Pathology, National Taiwan University Hospital Yun-Lin Branch, Yunlin, Taiwan
| | - Long-Wei Lin
- Department of Pathology, National Taiwan University Hospital Yun-Lin Branch, Yunlin, Taiwan
| | - Yueh-Min Lin
- Department of Pathology, Changhua Christian Hospital, Changhua, Taiwan
| | - Wei-Chou Lin
- Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan
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30
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Song Y, Liu S, Song L, Chen H, Bai M, Yan J, Luo T, Liu K, Sun L, Zhao Y. Case Report: Histiocytic Necrotizing Lymphadenitis (Kikuchi-Fujimoto Disease) Concurrent With Aseptic Meningitis. Front Neurol 2021; 12:565387. [PMID: 33959084 PMCID: PMC8093430 DOI: 10.3389/fneur.2021.565387] [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: 06/18/2020] [Accepted: 03/10/2021] [Indexed: 11/17/2022] Open
Abstract
Kikuchi–Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign, self-limiting disease characterized by local lymphadenopathy. Central nervous system involvement in KFD is extremely rare and remains a diagnostic challenge. Only 41 cases of aseptic meningitis associated with KFD have been reported worldwide, with just four cases (including our case) of KFD with meningitis as the first symptom. We report a case of KFD accompanied by aseptic meningitis with severely high intracranial pressure (400 mmH2O), increased white blood cell count (56 × 106/L), and moderately elevated protein level (0.52 g/L). This case is unique in the delayed appearance of lymphadenopathy. After 1 month of treatment with steroids, fever, headache, and lymphadenopathy gradually disappeared, and the result of cerebrospinal fluid examination gradually became normal. In conclusion, based on our case findings and our literature review on KFD with aseptic meningitis, a diagnosis of KFD should be considered when delayed appearance of lymphadenopathy is observed in patients with aseptic meningitis.
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Affiliation(s)
- Yanna Song
- Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China
| | - Shan Liu
- Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China
| | - Lei Song
- Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China
| | - Huaqiu Chen
- Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China
| | - Miaoshui Bai
- Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China
| | - Jinhua Yan
- Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China
| | - Tianfei Luo
- Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China
| | - Kangding Liu
- Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China
| | - Li Sun
- Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China
| | - Yang Zhao
- Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China
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31
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Müller CSL, Vogt T, Becker SL. Kikuchi-Fujimoto Disease Triggered by Systemic Lupus Erythematosus and Mycoplasma pneumoniae Infection-A Report of a Case and a Review of the Literature. Am J Dermatopathol 2021; 43:202-208. [PMID: 32809980 DOI: 10.1097/dad.0000000000001764] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
Abstract
ABSTRACT Kikuchi-Fujimoto disease (KFD) is a necrotizing histiocytic lymphadenitis that was described for the first time in 1972 in Japan. Its etiology is still not fully understood. It has been reported in association with many different agents, diseases, and triggering factors without any conclusive result. To the best of our knowledge, we report for the first time a case of KFD with systemic lupus erythematosus in a child in association with a polymerase chain reaction (PCR)-positive throat swab for Mycoplasma pneumoniae. Although difficult to prove, the acute M. pneumoniae infection might have served as a triggering event for the development of KFD in our case. We encourage further studies to investigate a potential relationship between KFD and M. pneumoniae, which should also use PCR-based testing for this pathogen in patients with KFD.
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Affiliation(s)
- Cornelia S L Müller
- Department of Dermatology, Saarland University Medical Center, Homburg/Saar, Germany
| | - Thomas Vogt
- Department of Dermatology, Saarland University Medical Center, Homburg/Saar, Germany
| | - Sören L Becker
- Institute of Medical Microbiology and Hygiene, Saarland University Medical Center, Homburg/Saar, Germany
- Swiss Tropical and Public Health Institute, Basel, Switzerland; and
- University of Basel, Basel, Switzerland
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32
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Pradhan S, Sirka CS, Dash G, Sahu K, Rout AN. A challenging case of Kikuchi-Fujimoto disease with systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis in a young man. Indian J Dermatol Venereol Leprol 2021; 87:78-82. [PMID: 33580926 DOI: 10.25259/ijdvl_893_18] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/01/2018] [Accepted: 07/01/2020] [Indexed: 11/04/2022]
Affiliation(s)
- Swetalina Pradhan
- Department of Dermatology, All India Institute of Medical Sciences, Patna, Bihar
| | - Chandra Sekhar Sirka
- Department of Dermatology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Gaurav Dash
- Department of Dermatology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Kananbala Sahu
- Department of Dermatology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
| | - Arpita Nibedita Rout
- Department of Dermatology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
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33
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Treichel AM, Teske N, Vandergriff T, Dominguez A, Mauskar MM, Nguyen KD. Kikuchi disease with cutaneous involvement complicated by features of hemophagocytic lymphohistiocytosis. J Cutan Pathol 2021; 48:343-346. [PMID: 33470433 DOI: 10.1111/cup.13720] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/28/2020] [Revised: 04/12/2020] [Accepted: 04/20/2020] [Indexed: 11/29/2022]
Affiliation(s)
- Alison M Treichel
- Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA
| | - Noelle Teske
- Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
| | - Travis Vandergriff
- Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
| | - Arturo Dominguez
- Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
| | - Melissa M Mauskar
- Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
| | - Khang D Nguyen
- Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
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Kim HY, Jo HY, Kim SH. Clinical and Laboratory Characteristics of Kikuchi-Fujimoto Disease According to Age. Front Pediatr 2021; 9:745506. [PMID: 34796153 PMCID: PMC8593182 DOI: 10.3389/fped.2021.745506] [Citation(s) in RCA: 20] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/22/2021] [Accepted: 10/12/2021] [Indexed: 01/23/2023] Open
Abstract
Background: Little information exists regarding the differences in the clinical and laboratory characteristics of Kikuchi-Fujimoto disease (KFD) according to age. Objective: To evaluate the clinical and laboratory characteristics of KFD according to age. Methods: The relevance of sex, age, clinical features, laboratory findings, courses, and follow-up results were retrospectively evaluated in patients diagnosed with KFD at Pusan National University Hospital between 2010 and 2020. Results: Eighty patients (46 children and 34 adults) with a mean age of 21.5 ± 11.8 years (range, 3-49 years) were included in the study. Those aged 10-19 years accounted for the largest number of patients (42.5%). Among children, the male sex ratio was higher, especially for patients aged ≤ 9 years. In adults, the female sex ratio was higher, especially for patients aged 20-29 years. Fever, tenderness in the lymph node, and skin rashes were more common in children, while myalgia and weight loss were more common in adults. In children, the recurrence rate was significantly higher among boys than among girls (15.8 vs. 0.0%, P = 0.001); lower platelet count and higher CRP levels were observed among boys than among girls. EBV and ANA positivity rates were higher in boys than in girls. In adults, the recurrence rate was significantly higher in women than in men (18.2 vs. 0.0%, P = 0.005). ANA positivity rates were higher in women than in men. Conclusion: The clinical features, laboratory findings, and recurrence of KFD may differ depending on age and sex. Clinicians should be aware of this.
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Affiliation(s)
- Hye-Young Kim
- Department of Pediatrics, Medical Research Institute, Pusan National University School of Medicine, Pusan National University Hospital, Busan, South Korea.,Department of Pediatrics, Pusan National University Hospital, Busan, South Korea
| | - Ha Young Jo
- Department of Pediatrics, Pusan National University Hospital, Busan, South Korea
| | - Seong Heon Kim
- Department of Pediatrics, Seoul National University Children's Hospital & College of Medicine, Seoul, South Korea
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35
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Duan W, Xiao ZH, Yang LG, Luo HY. Kikuchi's disease with hemophagocytic lymphohistiocytosis: A case report and literature review. Medicine (Baltimore) 2020; 99:e23500. [PMID: 33371072 PMCID: PMC7748204 DOI: 10.1097/md.0000000000023500] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/13/2019] [Accepted: 11/04/2020] [Indexed: 12/18/2022] Open
Abstract
INTRODUCTION Kikuchi's disease (KD) is a rare form of necrotizing lymphadenitis that rarely occurs in association with hemophagocytic lymphohistiocytosis (HLH) in children. PATIENT CONCERNS We report the case of a 4-year-5-month-old boy who suffered from fever, cervical lymphadenopathy, pancytopenia, hypertriglyceridemia, splenomegaly, low NK cell activity. DIAGNOSES A diagnosis of KD with HLH was made based on the results of biopsy of cervical lymph node and HLH-2004 trial guidelines. INTERVENTIONS The patient was treated with corticosteroids, cyclosporine, etoposide, continuous hemodiafiltration (HDF), and plasma exchange (PE). OUTCOMES He showed a complete response to therapy, and his condition gradually improved. He was discharged on day 45 after admission due to his good recovery status. CONCLUSION HLH can be associated with KD, especially in childhood, and may have an aggressive clinical course. Continuous HDF and PE and chemotherapy should be reserved for those patients who fail to respond to IVIG and corticosteroids.
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Halawa ARR, Ahmad MK, Nashwan AJ. An atypical presentation of Kikuchi-Fujimoto disease: A case report & literature review. Clin Case Rep 2020; 8:3515-3519. [PMID: 33363963 PMCID: PMC7752579 DOI: 10.1002/ccr3.3373] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/09/2020] [Revised: 08/01/2020] [Accepted: 08/24/2020] [Indexed: 11/09/2022] Open
Abstract
The authors urge clinicians to consider the possibility of Kikuchi-Fujimoto Disease associated with autoimmune hemolytic anemia with possible correlation with systemic lupus erythematosus in patients presented with lymphadenopathy and fever.
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Affiliation(s)
| | - Muayad K. Ahmad
- Department of MedicineHazm Mebaireek General Hospital (HMGH)Hamad Medical Corporation (HMC)DohaQatar
- Weill Cornell Medical College in Qatar (WCM‐Q)DohaQatar
| | - Abdulqadir J. Nashwan
- Hazm Mebaireek General Hospital (HMGH)Hamad Medical Corporation (HMC)DohaQatar
- University of Calgary in Qatar (UCQ)DohaQatar
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37
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Rare Presentation of Self-Limiting Kikuchi-Fujimoto Disease in Relapsing Nature. Case Rep Med 2020; 2020:9785104. [PMID: 33014067 PMCID: PMC7525287 DOI: 10.1155/2020/9785104] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/07/2020] [Revised: 09/13/2020] [Accepted: 09/16/2020] [Indexed: 11/21/2022] Open
Abstract
Background Kikuchi–Fujimoto disease (KFD) is a rare, benign, and self-limiting disease that is commonly associated with cervical lymphadenopathy and fever. The disease has a wide spectrum of clinical manifestations, and definitive diagnosis is based on the histological appearance in the excision biopsy of the lymph nodes. Recurrence of KFD is reported rarely. Case Presentation. A 56-year-old Iranian woman with a background history of thrombocytopenia presented with fever, malaise, loss of appetite, and weight loss with cervical lymphadenopathy. The excision biopsy of the cervical lymph nodes confirmed the diagnosis of KFD, and she made a full recovery with improvement of symptoms, regression of cervical lymph nodes, and normalization inflammatory markers. One year after remission, she presented with similar clinico-biochemical profile, and repeat biopsy confirmed KFD. Conclusion Although the rate of recurrence of the disease is very low, the treating physician should consider the possibility and confirm it histologically.
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Park SG, Koo HR, Jang K, Myung JK, Song CM, Ji YB, Park JS, Tae K. Efficacy of Ultrasound-Guided Needle Biopsy in the Diagnosis of Kikuchi-Fujimoto Disease. Laryngoscope 2020; 131:E1519-E1523. [PMID: 33002212 DOI: 10.1002/lary.29160] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/21/2020] [Revised: 08/06/2020] [Accepted: 09/21/2020] [Indexed: 12/19/2022]
Abstract
OBJECTIVES/HYPOTHESIS Ultrasound-guided fine-needle aspiration cytology (US-FNAC) is a well-established procedure performed to establish the diagnosis of Kikuchi-Fujimoto disease (KFD). Ultrasound-guided core needle biopsy (US-CNB) is an alternative diagnostic tool for KFD. However, the efficacy of US-CNB is not well evaluated. This study aimed to evaluate the efficacy of US-CNB and compare it with that of US-FNAC in the diagnosis of KFD. STUDY DESIGN Retrospective cohort study. METHODS We analyzed 170 patients who were diagnosed with KFD between January 2009 and May 2019. US-FNAC, US-CNB, and excisional biopsy were performed in 47, 114, and 9 patients, respectively. Diagnostic accuracies of US-FNAC and US-CNB were analyzed and compared. RESULTS Of the 170 patients, 45 and 125 were men and women, respectively. The mean age was 26.9 ± 9.1 years. The most common symptom was cervical lymphadenopathy, followed by fever, headache, and myalgia. The diagnosis of KFD was established primarily by US-FNAC in 21 (44.7%) of the 47 patients, by US-CNB in 109 (95.6%) of the 114 patients, and by excisional biopsy in all 9 patients. There was no specific major complication related to US-FNAC and US-CNB. CONCLUSION US-CNB can be considered safe and effective and used as the primary modality for the pathological diagnosis of KFD. LEVEL OF EVIDENCE 4. Laryngoscope, 131:E1519-E1523, 2021.
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Affiliation(s)
- Sang Gyu Park
- Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University, Seoul, Republic of Korea
| | - Hye Ryoung Koo
- Department of Radiology, College of Medicine, Hanyang University, Seoul, Republic of Korea
| | - Kiseok Jang
- Department of Pathology, College of Medicine, Hanyang University, Seoul, Republic of Korea
| | - Jae Kyung Myung
- Department of Pathology, College of Medicine, Hanyang University, Seoul, Republic of Korea
| | - Chang Myeon Song
- Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University, Seoul, Republic of Korea
| | - Yong Bae Ji
- Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University, Seoul, Republic of Korea
| | - Jeong Seon Park
- Department of Radiology, College of Medicine, Hanyang University, Seoul, Republic of Korea
| | - Kyung Tae
- Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University, Seoul, Republic of Korea
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Higaki M, Kanda T, Ando T, Tani R, Toratani S. Development of Kikuchi-Fujimoto disease after a cervical lymph node metastasis of mucoepidermoid carcinoma: a case report. Oral Maxillofac Surg 2020; 25:133-137. [PMID: 32856133 DOI: 10.1007/s10006-020-00900-4] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/12/2020] [Accepted: 08/24/2020] [Indexed: 11/29/2022]
Abstract
Kikuchi-Fujimoto disease (KFD) was first reported by Kikuchi and Fujimoto in 1972 as a rare disease with lymphadenitis of unknown etiology. KFD is characterized by the main symptoms of fever and enlarged cervical lymph nodes (LNs), which are similar to the features of other LN-associated diseases. Therefore, it is difficult to diagnose this condition. We report the case of a 24-year-old woman who presented with KFD after surgery to treat a mucoepidermoid carcinoma of the palate and dissection of the left neck. The patient presented with a fever and right cervical lymphadenopathy when she visited our department for a regular follow-up related to the mucoepidermoid carcinoma. The results of computed tomography and ultrasonography evaluations led to a clinical diagnosis of lymph node metastasis, and a right neck dissection was performed. However, the pathological tissue analysis did not suggest malignancy but showed necrosis and various cellular infiltrates. We made a diagnosis of KFD from these clinical and pathological features. KFD may be misdiagnosed as a LN-associated disease such as metastasis. Clinically, KFD should be considered in patients with head and neck cancer who present with cervical lymphadenopathy.
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Affiliation(s)
- Mirai Higaki
- Department of Oral and Maxillofacial Surgery, Hiroshima University Hospital, 1-2-3 Kasumi Minami-ku, Hiroshima, 734-8553, Japan.
| | - Taku Kanda
- Department of Oral and Maxillofacial Surgery, Hiroshima University Hospital, 1-2-3 Kasumi Minami-ku, Hiroshima, 734-8553, Japan
| | - Toshinori Ando
- Center of Oral Clinical Examination, Hiroshima University Hospital, Hiroshima, Japan
| | - Ryouji Tani
- Department of Oral and Maxillofacial Surgery, Hiroshima University Hospital, 1-2-3 Kasumi Minami-ku, Hiroshima, 734-8553, Japan
| | - Shigeaki Toratani
- Department of Molecular Oral Medicine and Maxillofacial Surgery, Division of Dentistry, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan
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Crawshaw H, Rachakonda KS, Kim L, Enno A, Jegatheesan T. Intensive care management of a rare case of pyrexia of unknown origin (Kikuchi-Fujimoto disease). Anaesth Intensive Care 2020; 48:318-323. [PMID: 32731741 DOI: 10.1177/0310057x20937314] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Kikuchi-Fujimoto disease is a rare, benign cause of necrotising lymphadenitis often presenting with fever. We describe a case of a 17-year-old boy with non-verbal autism presenting to our intensive care unit with prolonged fever of unknown cause. This case highlights the role of the intensive care unit in cases of diagnostic dilemma. The critical care community should be aware of Kikuchi-Fujimoto disease as although it is usually benign, it can rarely lead to acute airway compromise.
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Affiliation(s)
- Helena Crawshaw
- Department of General and Acute Care Medicine, Liverpool Hospital, Sydney, Australia
| | - Kanaka S Rachakonda
- Department of Intensive Care Medicine, Liverpool Hospital, Sydney, Australia
| | - Leah Kim
- Department of Intensive Care Medicine, Liverpool Hospital, Sydney, Australia
| | - Alar Enno
- Department of Anatomical Pathology, Liverpool Hospital, Sydney, Australia
| | - Thulasi Jegatheesan
- Department of General and Acute Care Medicine, Liverpool Hospital, Sydney, Australia
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Scott GD, Kumar J, Oak JS, Boyd SD, Raess PW, Gratzinger DA. Histology-Independent Signature Distinguishes Kikuchi-Fujimoto Disease/Systemic Lupus Erythematosus-Associated Lymphadenitis From Benign and Malignant Lymphadenopathies. Am J Clin Pathol 2020; 154:215-224. [PMID: 32367142 DOI: 10.1093/ajcp/aqaa036] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022] Open
Abstract
OBJECTIVES Kikuchi-Fujimoto disease (KFD) and systemic lupus erythematosus (SLE) are benign entities with histologic features that raise concern about malignancy and infection. We searched for a histology-independent KFD/SLE signature relying on only immunophenotype and basic clinical characteristics. METHODS A histology-independent KFD/SLE signature was generated using 975 excised lymph nodes with flow immunophenotyping, including 16 cases of KFD/SLE. This signature was then evaluated in 1,198 fine-needle aspiration (FNA) specimens. RESULTS The top flow cytometry discriminant for KFD/SLE was uniform CD38+ expression on CD19+ events. Immunohistochemistry demonstrated nodules of IgD+, IgM- B cells surrounding necrotizing and activated T-cell areas. A signature combining 6 flow cytometry criteria with age and sample site had a positive predictive value of 88% for KFD/SLE, which had a prevalence of 1.6%. All 4 signature-positive FNA cases with follow-up excision were KFD/SLE. At a second institution, 4 of 5 KFD/SLE cases passed the top discriminant. CONCLUSIONS A flow cytometry signature combined with age and biopsy site identifies KFD/SLE independent of histology, suggesting a shared immune composition and independently confirming that KFD/SLE represents a distinct entity. Unexpectedly, an IgD+CD38+ small B-cell population is a distinctive feature of KFD/SLE, suggesting a possible pathologic role for anergic/autoreactive B cells.
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Affiliation(s)
- Gregory D Scott
- Department of Pathology, Stanford University School of Medicine, Stanford, CA
| | - Jyoti Kumar
- Department of Pathology, Stanford University School of Medicine, Stanford, CA
| | - Jean S Oak
- Department of Pathology, Stanford University School of Medicine, Stanford, CA
| | - Scott D Boyd
- Department of Pathology, Stanford University School of Medicine, Stanford, CA
| | - Philipp W Raess
- Department of Pathology, Oregon Health and Science University, Portland
| | - Dita A Gratzinger
- Department of Pathology, Stanford University School of Medicine, Stanford, CA
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Alosaimi S, Hijazi B, Alhumidi A, Alsaif F. A Case of Kikuchi-Fujimoto Disease Associated with Erosive Lichen Planus. Cureus 2020; 12:e7312. [PMID: 32313753 PMCID: PMC7164705 DOI: 10.7759/cureus.7312] [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] [Indexed: 11/06/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a benign, self-limiting disorder with unknown etiology. The most frequent clinical manifestations include lymphadenopathy, fever, cutaneous lesions, arthritis, fatigue, and hepatosplenomegaly. Cutaneous manifestations of KFD, occurring in about one-third of patients, are typically non-specific, rarely presenting as symmetrically distributed lesions. The prevalence of erosive lichen planus in patients with KFD, as of this date, is unknown with no previously reported cases describing an association between the two conditions. In the following case report, we report a case presenting with bilateral symmetrical erosive lichen planus of the heel associated with KFD as being a possible, rather novel, cutaneous manifestation.
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Affiliation(s)
- Shoroq Alosaimi
- Department of Dermatology, College of Medicine, Majmaah University, Majmaah, SAU
| | | | - Ahmed Alhumidi
- Department of Pathology, College of Medicine, King Saud University, Riyadh, SAU
| | - Fahad Alsaif
- Department of Dermatology, College of Medicine, King Saud University, Riyadh, SAU
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Park JE, Ryu YJ, Kim JY, Kim YH, Park JY, Lee H, Choi HS. Cervical lymphadenopathy in children: a diagnostic tree analysis model based on ultrasonographic and clinical findings. Eur Radiol 2020; 30:4475-4485. [PMID: 32189052 DOI: 10.1007/s00330-020-06794-w] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/28/2019] [Revised: 02/26/2020] [Accepted: 03/05/2020] [Indexed: 12/14/2022]
Abstract
OBJECTIVES To establish a diagnostic tree analysis (DTA) model based on ultrasonography (US) findings and clinical characteristics for differential diagnosis of common causes of cervical lymphadenopathy in children. METHODS A total of 242 patients (131 boys, 111 girls; mean age, 11.2 ± 0.3 years; range, 1 month-18 years) with pathologically confirmed Kikuchi disease (n = 127), reactive hyperplasia (n = 64), lymphoma (n = 24), or suppurative lymphadenitis (n = 27) who underwent neck US were included. US images were retrospectively reviewed to assess lymph node (LN) characteristics, and clinical information was collected from patient records. DTA models were created using a classification and regression tree algorithm on the basis of US imaging and clinical findings. The patients were randomly divided into training (70%, 170/242) and validation (30%, 72/242) datasets to assess the diagnostic performance of the DTA models. RESULTS In the DTA model based on all predictors, perinodal fat hyperechogenicity, LN echogenicity, and short diameter of the largest LN were significant predictors for differential diagnosis of cervical lymphadenopathy (overall accuracy, 85.3% and 83.3% in the training and validation datasets). In the model based on categorical parameters alone, perinodal fat hyperechogenicity, LN echogenicity, and loss of fatty hilum were significant predictors (overall accuracy, 84.7% and 86.1% in the training and validation datasets). CONCLUSIONS Perinodal fat hyperechogenicity, heterogeneous echotexture, short diameter of the largest LN, and loss of fatty hilum were significant US findings in the DTA for differential diagnosis of cervical lymphadenopathy in children. KEY POINTS • Diagnostic tree analysis model based on ultrasonography and clinical findings would be helpful in differential diagnosis of pediatric cervical lymphadenopathy. • Significant predictors were perinodal fat hyperechogenicity, heterogeneous echotexture, short diameter of the largest LN, and loss of fatty hilum.
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Affiliation(s)
- Ji Eun Park
- Department of Radiology, Seoul National University Bundang Hospital, 82 Gumi-ro 173 Beon-gil, Bundang-gu, Seongnam, 13620, South Korea
| | - Young Jin Ryu
- Department of Radiology, Seoul National University Bundang Hospital, 82 Gumi-ro 173 Beon-gil, Bundang-gu, Seongnam, 13620, South Korea. .,Department of Radiology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.
| | - Ji Young Kim
- Department of Radiology, Seoul National University Bundang Hospital, 82 Gumi-ro 173 Beon-gil, Bundang-gu, Seongnam, 13620, South Korea.,Department of Radiology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul, 03080, South Korea
| | - Young Hoon Kim
- Department of Radiology, Seoul National University Bundang Hospital, 82 Gumi-ro 173 Beon-gil, Bundang-gu, Seongnam, 13620, South Korea.,Department of Radiology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul, 03080, South Korea
| | - Ji Young Park
- Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, South Korea
| | - Hyunju Lee
- Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, South Korea.,Department of Pediatrics, Seoul National University College of Medicine, Seoul, South Korea
| | - Hyoung Soo Choi
- Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, South Korea.,Department of Pediatrics, Seoul National University College of Medicine, Seoul, South Korea
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Ramachandran D, Venkitakrishnan R, Augustine J, Cleetus M. Spontaneous resolution of persistent lymphadenitis: a case of Kikuchi–Fujimoto disease. THE EGYPTIAN JOURNAL OF BRONCHOLOGY 2019. [DOI: 10.4103/ejb.ejb_26_19] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
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Fernandez-Flores A. La biopsia cutánea en el contexto de la enfermedad sistémica. ACTAS DERMO-SIFILIOGRAFICAS 2019; 110:710-727. [DOI: 10.1016/j.ad.2019.02.012] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2018] [Revised: 02/11/2019] [Accepted: 02/12/2019] [Indexed: 02/07/2023] Open
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Sangiorgio V, Veloza L, Galvis K, López M, Frigola G, Campo E, Balagué O. Kikuchi-Fujimoto disease and breast implants: is there a relationship? Haematologica 2019; 104:e581-e584. [PMID: 31582552 DOI: 10.3324/haematol.2019.229831] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022] Open
Affiliation(s)
| | - Luis Veloza
- Haematopathology Unit, Pathology Department, Hospital Clinic of Barcelona, University of Barcelona, Villarroel, Barcelona, Spain and
| | - Karen Galvis
- Department of Pathology and Laboratory Medicine, Fundación Santa Fe de Bogotá University Hospital, Bogotá, Colombia
| | - Mónica López
- Haematopathology Unit, Pathology Department, Hospital Clinic of Barcelona, University of Barcelona, Villarroel, Barcelona, Spain and
| | - Gerard Frigola
- Haematopathology Unit, Pathology Department, Hospital Clinic of Barcelona, University of Barcelona, Villarroel, Barcelona, Spain and
| | - Elias Campo
- Haematopathology Unit, Pathology Department, Hospital Clinic of Barcelona, University of Barcelona, Villarroel, Barcelona, Spain and
| | - Olga Balagué
- Haematopathology Unit, Pathology Department, Hospital Clinic of Barcelona, University of Barcelona, Villarroel, Barcelona, Spain and
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Phenytoin-associated necrotising lymphadenitis mimicking Kikuchi disease. Pathology 2019; 51:650-653. [DOI: 10.1016/j.pathol.2019.04.010] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2019] [Revised: 03/25/2019] [Accepted: 04/07/2019] [Indexed: 11/20/2022]
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Batton E, Alali M, Hageman JR, Parilla M, Yu KOA. Kikuchi-Fujimoto Disease in Children: An Important Diagnostic Consideration for Cervical Lymphadenitis. Pediatr Ann 2019; 48:e406-e411. [PMID: 31610000 DOI: 10.3928/19382359-20190920-01] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a rare, benign, and self-limited disease that causes lymphadenopathy and has a characteristic histological appearance. The etiology of this disease is unknown, but a possible infectious trigger has been hypothesized. In the adult population this disease is more common in females; however, in the pediatric population it is more common in males. Descriptions in the pediatric literature are lacking, particularly in the United States. The authors report three cases of pediatric KFD that presented at the same institution in a 9-month time period. All three patients were male and of non-Asian descent who were diagnosed with KFD by histopathologic specimen after presenting with unilateral cervical lymphadenitis. Each patient had additional laboratory evidence of a possible bacterial infection at the time of diagnosis. These three cases highlight the importance of considering KFD early when a pediatric patient presents with unilateral cervical lymphadenitis. The authors discuss the epidemiology, etiology, clinical manifestations, diagnostic approaches, and treatment of KFD. [Pediatr Ann. 2019;48(10):e406-e411.].
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Chen LC, Wang CJ, Chang YC, Shie SS, Lin TY, Hsieh YC, Arthur Huang KY, Kuo CY, Chiu CH, Huang YC, Chen CJ. Distribution of lymphadenopathy in patients with Kikuchi disease. JOURNAL OF MICROBIOLOGY, IMMUNOLOGY, AND INFECTION = WEI MIAN YU GAN RAN ZA ZHI 2019; 54:299-304. [PMID: 31615722 DOI: 10.1016/j.jmii.2019.08.016] [Citation(s) in RCA: 19] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 02/24/2019] [Revised: 07/17/2019] [Accepted: 08/13/2019] [Indexed: 11/28/2022]
Abstract
BACKGROUND Cervical lymphadenopathy is among the cardinal manifestations of Kikuchi disease (KD). The incidences and locations of extra-cervical lymph nodes (LNs) involvement in KD have not been comprehensively reported. METHODS From 2003 to 2016, 60 patients with pathologically confirmed KD and with computed tomography and/or whole-body inflammation scans at diagnosis were retrospectively identified. The locations, sizes and characteristics of all affected LNs were analyzed by extensive review of the image studies. The clinical and laboratory parameters were abstracted from medical records and the associations with extra-cervical LNs involvement were identified. RESULTS Female accounted for 35 (58.3%) patients and the median age of all patients was 21.3 years (ranges, 3-64 years). Of 59 patients with evaluable neck images, 42 (71.2%) and 16 (27.1%) patients presented with unilateral and bilateral nodal involvement, respectively, with the most common locations at level II, III and IV by Som's classification. The largest LNs appeared most commonly in level II. The incidences of extra-cervical lymphadenopathy in abdomen, pelvis, inguina, axilla and mediastinum with available images were respectively 52.9% (9/17), 47.1% (8/17), 41.2% (7/17), 30.6% (11/36) and 14.3% (8/56). When compared to cases with solitary cervical lymphadenopathy, the cases with extra-cervical lymphadenopathy had significantly greater incidences of bilateral cervical lymphadenopathy (P = .0379) and leukopenia (P = .0173). CONCLUSION Unilateral cervical lymphadenopathy was the most frequent form of LNs involvement of KD. Extra-cervical lymphadenopathy was not uncommon and was associated with the appearance of bilateral distribution of cervical LNs and leukopenia.
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Affiliation(s)
- Lung-Chiung Chen
- Division of Pediatric Infectious Diseases, Department of Pediatrics, Chang Gung Memorial Hospital, 333 Taoyuan, Taiwan; Department of Pediatrics, Taoyuan Armed Forces General Hospital, 325 Taoyuan, Taiwan
| | - Chao-Jan Wang
- Department of Radiology, Chang Gung Memorial Hospital, 333 Taoyuan, Taiwan
| | - Yu-Chuan Chang
- Department of Nuclear Medicine, Chang Gung Memorial Hospital, 333 Taoyuan, Taiwan; Department of Medical Imaging and Radiological Sciences, Chang Gung University, 333 Taoyuan, Taiwan
| | - Shian-Sen Shie
- Division of Infectious Diseases, Department of Internal Medicine, Chang Gung Memorial Hospital, 333 Taoyuan, Taiwan; School of Medicine, College of Medicine, Chang Gung University, 333 Taoyuan, Taiwan
| | - Tzou-Yien Lin
- Division of Pediatric Infectious Diseases, Department of Pediatrics, Chang Gung Memorial Hospital, 333 Taoyuan, Taiwan; School of Medicine, College of Medicine, Chang Gung University, 333 Taoyuan, Taiwan
| | - Yu-Chia Hsieh
- Division of Pediatric Infectious Diseases, Department of Pediatrics, Chang Gung Memorial Hospital, 333 Taoyuan, Taiwan; School of Medicine, College of Medicine, Chang Gung University, 333 Taoyuan, Taiwan
| | - Kuan-Ying Arthur Huang
- Division of Pediatric Infectious Diseases, Department of Pediatrics, Chang Gung Memorial Hospital, 333 Taoyuan, Taiwan; School of Medicine, College of Medicine, Chang Gung University, 333 Taoyuan, Taiwan
| | - Chen-Yen Kuo
- Division of Pediatric Infectious Diseases, Department of Pediatrics, Chang Gung Memorial Hospital, 333 Taoyuan, Taiwan; School of Medicine, College of Medicine, Chang Gung University, 333 Taoyuan, Taiwan
| | - Cheng-Hsun Chiu
- Division of Pediatric Infectious Diseases, Department of Pediatrics, Chang Gung Memorial Hospital, 333 Taoyuan, Taiwan; School of Medicine, College of Medicine, Chang Gung University, 333 Taoyuan, Taiwan
| | - Yhu-Chering Huang
- Division of Pediatric Infectious Diseases, Department of Pediatrics, Chang Gung Memorial Hospital, 333 Taoyuan, Taiwan; School of Medicine, College of Medicine, Chang Gung University, 333 Taoyuan, Taiwan
| | - Chih-Jung Chen
- Division of Pediatric Infectious Diseases, Department of Pediatrics, Chang Gung Memorial Hospital, 333 Taoyuan, Taiwan; School of Medicine, College of Medicine, Chang Gung University, 333 Taoyuan, Taiwan.
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