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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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Cao Dinh H, Doan DM, Tran KQ, Tran TT, Nguyen SL. Kikuchi-Fujimoto disease co-occuring with Hashimoto thyroiditis: A case report and literature review. Ann Clin Biochem 2025; 62:75-80. [PMID: 39256357 DOI: 10.1177/00045632241280595] [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: 09/12/2024]
Abstract
We introduce a 16-year-old female who presented with tender cervical lymphadenopathy, prolonged fever, and hypothyroidism. After excluding common causes of fever of unknown origin, a surgical biopsy of cervical lymph nodes revealed Kikuchi-Fujimoto disease. The patient showed improvement with a short-term course of NSAIDs. An increased titre of thyroperoxidase antibody led to a diagnosis of Hashimoto's thyroiditis during stable condition. This report underscores the importance of considering Kikuchi-Fujimoto disease in the differential diagnosis of prolonged fever of unknown origin with lymphadenopathy and highlights the association with Hashimoto's thyroiditis, advocating for vigilance regarding hypothyroidism in long-term follow-up after Kikuchi-Fujimoto disease recovery.
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Affiliation(s)
- Hung Cao Dinh
- Pham Ngoc Thach University of Medicine, Ho Chi Minh City, Vietnam
- Nguyen Tat Thanh University, Ho Chi Minh City, Vietnam
- Nguyen Tri Phuong Hospital, Ho Chi Minh City, Vietnam
| | - Dung Manh Doan
- University of Medicine and Pharmacy at Ho Chi Minh City, Ho Chi Minh City, Vietnam
| | - Khanh Quang Tran
- Nguyen Tri Phuong Hospital, Ho Chi Minh City, Vietnam
- University of Medicine and Pharmacy at Ho Chi Minh City, Ho Chi Minh City, Vietnam
| | - Trung The Tran
- University of Medicine and Pharmacy at Ho Chi Minh City, Ho Chi Minh City, Vietnam
| | - Si Luc Nguyen
- Pham Ngoc Thach University of Medicine, Ho Chi Minh City, Vietnam
- Nguyen Tri Phuong Hospital, Ho Chi Minh City, Vietnam
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Daoo BT, Alhayek S, Alakhrass D, Shibani M, Khallouf MS. Kikuchi-Fujimoto disease: 6 years old boy rare case in Syria. Oxf Med Case Reports 2025; 2025:omae172. [PMID: 39834884 PMCID: PMC11742175 DOI: 10.1093/omcr/omae172] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2024] [Revised: 09/25/2024] [Accepted: 10/30/2024] [Indexed: 01/22/2025] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare condition first identified in Japan in 1972. It typically presents with high fever and lymph node swelling, and may be linked to autoimmune conditions or viral infections. A 6-year-old boy presented with cervical enlargement and recurrent high fever. Physical examination revealed enlarged lymph nodes in the neck, axillary, and inguinal regions, along with hepatosplenomegaly. CT scans confirmed widespread lymphadenopathy, and blood tests showed elevated white blood cell count and C-reactive protein levels. Although lymphoma was suspected, biopsies indicated atypical lymphadenitis. Despite a positive Epstein-Barr virus test, no treatment was effective. Further lymph node biopsies ruled out lymphoma, EBV, and TB, ultimately confirming Kikuchi-Fujimoto Disease.
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Affiliation(s)
- Baseel Tawfik Daoo
- Department of Pediatrics at Al-Baath Hospital, Al-Baath University, Al-Wehda, Homs, Syria
| | - Shahd Alhayek
- Faculty of Medicine, Syrian Private University, M5, Al Kiswah, Damascus, Syria
| | - Dana Alakhrass
- Faculty of Medicine, Al-Baath University, Al-Wehda, Homs, Syria
| | - Mosa Shibani
- Faculty of Medicine, Syrian Private University, M5, Al Kiswah, Damascus, Syria
- School of Health and Wellbeing, University of Glasgow, 90 Byres Rd, Glasgow, United Kingdom
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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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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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Brown MP, Scott AR. Tender Cervical Lymphadenopathy in a Pediatric Patient. JAMA Otolaryngol Head Neck Surg 2024; 150:77-78. [PMID: 37971753 DOI: 10.1001/jamaoto.2023.3669] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2023]
Abstract
An 11-year-old boy presented with enlarged and tender cervical lymph nodes that showed areas of central hypodensity and necrosis with histiocytes and an absence of neutrophils. What is your diagnosis?
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Affiliation(s)
- Morgan P Brown
- University of New England College of Osteopathic Medicine, Biddeford, Maine
| | - Andrew R Scott
- Department of Otolaryngology-Head and Neck Surgery, Tufts Medical Center, Boston, Massachusetts
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Yousefi M, Rukerd MRZ, Binafar H, Shoaie S, Mirkamali H, Pourzand P, Kaveh R. The co-occurrence of Kikuchi-Fujimoto disease and systemic lupus erythematosus: a case report. J Med Case Rep 2023; 17:448. [PMID: 37884991 PMCID: PMC10605312 DOI: 10.1186/s13256-023-04186-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/22/2023] [Accepted: 09/20/2023] [Indexed: 10/28/2023] Open
Abstract
BACKGROUND Kikuchi-Fujimoto disease is an uncommon systemic disease that mostly affects young women. Kikuchi-Fujimoto disease typically manifests as necrotizing lymphadenopathy, which frequently follows by a fever; however, Kikuchi-Fujimoto disease occurs rarely in extranodal regions. One of the most important accompaniments of Kikuchi-Fujimoto disease is its connection with autoimmune diseases such as systemic lupus erythematosus. This case presents a simultaneous occurrence of Kikuchi-Fujimoto disease with liver involvement and systemic lupus erythematosus in a young female patient. CASE PRESENTATION We present a rare case of a 20-year-old white woman who presented with fever, joint pains, myalgia, and shortness of breath. Initial hospitalization and treatment for fever of unknown origin did not yield improvement. Physical examination revealed cervical and supraclavicular lymphadenopathy, and laboratory investigations showed abnormal blood counts, elevated inflammatory markers, and positive autoimmune serologies. Imaging studies revealed bilateral pleural effusion and liver lesions. Lymph node biopsy confirmed the diagnosis of Kikuchi-Fujimoto disease, and liver biopsy showed extranodal involvement. The patient was diagnosed with Kikuchi-Fujimoto disease-associated systemic lupus erythematosus and treated with hydroxychloroquine and corticosteroids. The patient showed gradual resolution of symptoms and lymphadenopathy with treatment. CONCLUSION Kikuchi-Fujimoto disease is a rare systemic condition primarily impacting young females. It is characterized by necrotizing lymphadenopathy, often accompanied by fever. Although Kikuchi-Fujimoto disease is predominantly seen in the lymph nodes, occurrences in non-nodal areas are infrequent. When diagnosing Kikuchi-Fujimoto disease, it is essential to screen patients for systemic lupus erythematosus. In this particular case, we observed liver involvement along with the presence of both Kikuchi-Fujimoto disease and systemic lupus erythematosus.
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Affiliation(s)
- Maysam Yousefi
- Infectious Diseases Research Center of Tropical and Infectious Diseases, Kerman University of Medical Sciences, Kerman, Iran
| | - Mohammad Rezaei Zadeh Rukerd
- Gastroenterology and Hepatology Research Center, Institute of Basic and Clinical Physiology Sciences, Kerman University of Medical Sciences, Kerman, Iran
| | - Hanieh Binafar
- Student Research Committee, School of Medicine, Kerman University of Medical Sciences, Kerman, Iran
| | - Sahar Shoaie
- Department of Internal Medicine, Afzalipour Hospital, Kerman University of Medical Sciences, Kerman, Iran
| | - Hanieh Mirkamali
- Student Research Committee, School of Medicine, Kerman University of Medical Sciences, Kerman, Iran
| | - Pouria Pourzand
- Department of Emergency Medicine, School of Medicine, University of Medicine, Minneapolis, USA
| | - Roxana Kaveh
- Student Research Committee, School of Medicine, Kerman University of Medical Sciences, Kerman, Iran.
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Mahajan VK, Sharma V, Sharma N, Rani R. Kikuchi-Fujimoto disease: A comprehensive review. World J Clin Cases 2023; 11:3664-3679. [PMID: 37383134 PMCID: PMC10294163 DOI: 10.12998/wjcc.v11.i16.3664] [Citation(s) in RCA: 19] [Impact Index Per Article: 9.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/27/2022] [Revised: 03/29/2023] [Accepted: 04/27/2023] [Indexed: 06/02/2023] Open
Abstract
Kikuchi-Fujimoto disease, a rare form of necrotizing lymphadenitis, is an uncommon, benign, self-limiting disorder of obscure etiology. It affects mostly young adults of both genders. Clinically, it presents with fever and lymphadenopathy of a firm to rubbery consistency frequently involving cervical lymph nodes while weight loss, splenomegaly, leucopenia, and elevated erythrocyte sedimentation rate feature in severely affected patients. Cutaneous involvement occurs in about 30%-40% of cases as facial erythema and nonspecific erythematous papules, plaques, acneiform or morbilliform lesions of great histologic heterogeneity. Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an obscure and complex relationship as systemic lupus erythematosus may occasionally precede, develop subsequently, or sometimes be associated concurrently with Kikuchi-Fujimoto disease. It is often mistaken for non-Hodgkin lymphoma while lupus lymphadenitis, cat-scratch disease, Sweet's syndrome, Still's disease, drug eruptions, infectious mononucleosis, and viral or tubercular lymphadenitis are other common differentials. Fine needle aspiration cytology mostly has features of nonspecific reactive lymphadenitis and immunohistochemistry studies usually show variable features of uncertain diagnostic value. Since its diagnosis is exclusively from histopathology, it needs to be evaluated more carefully; an early lymph node biopsy will obviate the need for unnecessary investigations and therapeutic trials. Its treatment with systemic corticosteroids, hydroxychloroquine, or antimicrobial agents mostly remains empirical. The article reviews clinicoepidemiological, diagnostic, and management aspects of KFD from the perspective of practicing clinicians.
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Affiliation(s)
- Vikram K Mahajan
- Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
| | - Vikas Sharma
- Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
| | - Neeraj Sharma
- Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
| | - Ritu Rani
- Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
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Harrison J, Sukumaran S, Vijayan V. Systemic Lupus Erythematosus Lymphadenopathy Presenting as Kikuchi-Fujimoto Disease in an Adolescent. Cureus 2023; 15:e35304. [PMID: 36968890 PMCID: PMC10038115 DOI: 10.7759/cureus.35304] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/21/2023] [Indexed: 02/24/2023] Open
Abstract
Systemic lupus erythematosus is a multisystem autoimmune disorder with a highly heterogeneous clinical presentation. The clinical phenotype varies from mild cutaneous and musculoskeletal manifestations to neurological involvement. Lymphadenopathy is a frequent manifestation of SLE, but the association is often not recognized, as lymphadenopathy is not a criterion for diagnosis. An unusual and seldom reported mimicker of lupus lymphadenitis is Kikuchi-Fujimoto Disease. This is a rare self-limiting disease of young adult females that presents with lymphadenopathy, fever, and systemic symptoms. Lupus lymphadenitis and KFD share some common clinical and pathologic features; but distinguishing between those two diseases can be challenging. We describe a 16-year-old Hispanic female who presented with axillary lymphadenopathy and was initially diagnosed with KFD based on an excisional lymph node biopsy; but later met the criteria for the 2019 European League Against Rheumatism/American College of Rheumatology classification criteria SLE. This case highlights the need for clinicians to be aware that patients with SLE may present with lymphadenopathy and to consider the association between Kikuchi disease and SLE to prevent misdiagnosis and allow for timely treatment to avoid complications.
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Azzam M, Helali H, Sharif E, Musa R, Hamdi M, Hotait H, Aldirawi M, Alhamdani S, Yavuz L. Kikuchi Disease in Children: A Report of Two Cases. Cureus 2023; 15:e35160. [PMID: 36949986 PMCID: PMC10028400 DOI: 10.7759/cureus.35160] [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: 02/15/2023] [Indexed: 02/21/2023] Open
Abstract
Kikuchi disease (KD) is a benign self-limiting rare disease with unknown etiology. Prolonged fever with tender neck lymphadenitis is the most common presentation. Blood tests are not specific, and the final diagnosis is by biopsy. We describe two patients, ages seven and twelve years, who presented with fever and neck lymphadenitis. Both cases received antibiotics for more than two weeks without improvement. Blood work showed high inflammatory markers. The manifestation of the second case overlapped with Hashimoto's disease. The later diagnosis was confirmed by lymph node (LN) biopsy.
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Affiliation(s)
- Motaz Azzam
- Pediatrics, Al Jalila Children's Speciality Hospital, Dubai, ARE
| | - Hadi Helali
- Pediatrics, Al Jalila Children's Speciality Hospital, Dubai, ARE
| | - Elsade Sharif
- Pediatric Rheumatology, Al Jalila Children's Speciality Hospital, Dubai, ARE
| | - Rehab Musa
- Hospital Medicine, Al Jalila Children's Speciality Hospital, Dubai, ARE
| | - Moataz Hamdi
- Hospital Medicine, Al Jalila Children's Speciality Hospital, Dubai, ARE
| | | | - Mohammed Aldirawi
- Hospital Medicine, Al Jalila Children's Speciality Hospital, Dubai, ARE
| | - Sarmad Alhamdani
- Pediatrics, Al Jalila Children's Speciality Hospital, Dubai, ARE
| | - Lemis Yavuz
- Hospital Medicine, Al Jalila Children's Speciality Hospital, Dubai, ARE
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Shen Z, Ling J, Zhu X, Yang J, He T. Macrophage activation syndrome in children with Kikuchi-Fujimoto disease. Pediatr Rheumatol Online J 2023; 21:10. [PMID: 36698152 PMCID: PMC9875483 DOI: 10.1186/s12969-023-00788-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2022] [Accepted: 01/11/2023] [Indexed: 01/26/2023] Open
Abstract
BACKGROUND Kikuchi-Fujimoto disease (KFD) is typically a benign, self-limiting inflammatory disease. However, some patients may have a prolonged or recurrent disease course, or present with life-threatening complications such as macrophage activation syndrome (MAS). In this study, we aimed to describe the incidence and clinical features of MAS in KFD and to access potential laboratory markers for the diagnosis of KFD-associated MAS. METHODS Patients with KFD were retrospectively enrolled from January 2015 to November 2021 at Shenzhen Children's Hospital. Clinical data were collected from inpatient or outpatient medical records. Data collected included clinical manifestations, laboratory and imaging findings, treatment, and clinical outcomes. Data were analyzed using GraphPad Prism 8.0 statistical software (GraphPad Software Inc., La Jolla, CA, USA). A receiver operating characteristic (ROC) curve analysis was further performed to access the potential predictors for the KFD-MAS diagnosis. RESULTS Of 58 patients with a histological diagnosis of KFD, 15 (25.9%) patients had MAS. Compared to patients without MAS, patients with KFD-MAS presented with a higher proportion of skin rash (26.7%, p = 0.01), glucocorticoid treatment (80%, p = 0.003), and disease recurrence (33.3%, p = 0.04). KFD-MAS patients had lower absolute peripheral white blood cell (WBC, p = 0.02), platelet (p = 0.002), serum albumin levels (p = 0.01), and lymphocyte count (p < 0.0001), and higher lactate dehydrogenase (LDH) levels (p < 0.0001). ROC curve analysis showed that the cutoff values of absolute lymphocyte count, an absolute platelet count, serum albumin level, and serum LDH level for KFD-MAS diagnosis were < 1235/μL, < 171 × 106/μL, < 35.6 g/L, and > 679 IU/mL, respectively. CONCLUSIONS The presence of KFD-MAS in children may be more common than previously expected, especially in those with skin rash. KFD-MAS may be associated with a higher recurrence rate. An extremely elevated serum LDH level and moderate to severe lymphopenia may be useful diagnostic markers for MAS in KFD. TRIAL REGISTRATION Not applicable; this was a retrospective study.
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Affiliation(s)
- Zixuan Shen
- grid.452787.b0000 0004 1806 5224Department of Rheumatology and Immunology, Shenzhen Children’s Hospital, 7019 Yitian Road, Shenzhen, 518038 China
| | - Jiayun Ling
- grid.452787.b0000 0004 1806 5224Department of Rheumatology and Immunology, Shenzhen Children’s Hospital, 7019 Yitian Road, Shenzhen, 518038 China
| | - Xiaona Zhu
- grid.452787.b0000 0004 1806 5224Department of Rheumatology and Immunology, Shenzhen Children’s Hospital, 7019 Yitian Road, Shenzhen, 518038 China
| | - Jun Yang
- grid.452787.b0000 0004 1806 5224Department of Rheumatology and Immunology, Shenzhen Children’s Hospital, 7019 Yitian Road, Shenzhen, 518038 China
| | - Tingyan He
- Department of Rheumatology and Immunology, Shenzhen Children's Hospital, 7019 Yitian Road, Shenzhen, 518038, China.
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Baek JY, Kang JM, Lee JY, Lim SM, Ahn JG. Comparison of Clinical Characteristics and Risk Factors for Recurrence of Kikuchi-Fujimoto Disease Between Children and Adult. J Inflamm Res 2022; 15:5505-5514. [PMID: 36172546 PMCID: PMC9512633 DOI: 10.2147/jir.s378790] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2022] [Accepted: 09/08/2022] [Indexed: 11/23/2022] Open
Abstract
Purpose Kikuchi-Fujimoto disease (KFD) is a rare, benign, and self-limited disease, characterized by cervical lymphadenopathy and fever. Herein, we analyzed the differences in its clinical manifestations and risk factors for recurrence between children and adults. Patients and Methods We retrospectively reviewed the medical records of patients diagnosed with KFD at a tertiary referral hospital between 2005 and 2019. Patients were divided into two groups based on their age: children (<19 years) and adults (≥19 years). Results During the 14-year study period, 127 patients were diagnosed with KFD. Among these, 34 (26.8%) were children and 93 (73.2%) were adults. The fever duration was longer and the frequency of myalgia was higher in adults than in children; however, no other significant symptomatic differences were noted between the two groups. Lymph node evaluation was mainly performed using ultrasound in children (61.8%) and computed tomography in adults (78.5%). Moreover, the frequency of antibiotic use was higher in children than in adults (76.5% vs 54.8%, P = 0.027). In adults, multivariable logistic regression analysis revealed anti-nuclear antibody (ANA) positivity (titer ≥1:80) as a risk factor for recurrence (odds ratio: 7.813; 95% confidence interval = 1.818-33.333; P = 0.006). Conclusion The clinical features of KFD in children and adults were similar; however, the preferred imaging study and frequency of antibiotic use differed significantly between the two groups. Furthermore, in adults, ANA positivity was associated with KFD recurrence. Thus, patients with KFD who present with ANA positivity at diagnosis will benefit from a regular follow-up for monitoring KFD recurrence.
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Affiliation(s)
- Jee Yeon Baek
- Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea
| | - Ji-Man Kang
- Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea.,Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Korea
| | - Ji Young Lee
- Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea
| | - Sung Min Lim
- Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea
| | - Jong Gyun Ahn
- Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, Korea.,Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Korea
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14
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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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15
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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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16
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Abstract
BACKGROUND Kikuchi disease (KD) is a rare and generally benign condition of uncertain etiology that presents with nonspecific symptoms including fever and cervical lymphadenopathy. Clinical presentations can vary. Here, we present an atypical case of KD in a 10-year-old girl, as well as an updated literature review of the clinical presentation, laboratory features and management of KD in children. METHODS Studies (published up until February 2020) were identified through searches of PubMed using the following search items: Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis or Kikuchi disease. Our primary search resulted in 1117 publications. A total of 34 publications with a total of 670 patients were included in the final analysis. RESULTS All children present with lymphadenopathy. Almost all (96.3%) have cervical lymphadenopathy. Fever is recorded in the majority of children (77.1%). Analysis of laboratory features found that the majority of children have leukopenia (56.0%) and a raised erythrocyte sedimentation rate (56.0%). Over 30% have a raised C-reactive protein and anemia. Other features such as leukocytosis, thrombocytopenia and antinuclear antibodies positivity are less common. KD is mostly self-limiting, but steroids, hydroxychloroquine and intravenous immunoglobulin are used in protracted courses. Their efficacy has yet to be established in clinical trials. CONCLUSIONS The presentation of KD is variable, and there is no specific set of symptoms or laboratory features that reliably establishes the diagnosis. Thus, histopathology is crucial. Definitive evaluation and establishment of effective treatments will require future prospective research studies for a more comprehensive description of the clinical course and effects of treatment. Given the rarity of the disease, this will have to be performed in collaborative consortia.
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Affiliation(s)
- Ahmed Abdu
- From the Oxford University Medical School, University of Oxford, Oxford, United Kingdom
| | - Dasja Pajkrt
- Department of Pediatric Infectious Diseases, Emma Children's Hospital, Amsterdam UMC, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands
| | - Else M Bijker
- Oxford Vaccine Group, Department of Paediatrics, University of Oxford and the NIHR Oxford Biomedical Research Centre, Oxford, United Kingdom
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17
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Hoan L, Minh Hang L, Tuan Linh L, Thi Tra My T, Ngoc Minh T, Duc Thinh D, Ngoc Cuong N. A Rare Case of Kikuchi-Fujimoto Disease in a Young Female Patient. AMERICAN JOURNAL OF CASE REPORTS 2021; 22:e933377. [PMID: 34802030 PMCID: PMC8614063 DOI: 10.12659/ajcr.933377] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Patient: Female, 25-year-old
Final Diagnosis: Kikuchi-Fujimoto disease
Symptoms: Neck pain • palpable mass neck
Medication: —
Clinical Procedure: —
Specialty: Pathology • Pulmonology • Radiology
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Affiliation(s)
- Le Hoan
- Department of Respiratory Medicine, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Le Minh Hang
- Department of Respiratory Medicine, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Le Tuan Linh
- Diagnostic Imaging and Interventional Radiology Center, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Thieu Thi Tra My
- Diagnostic Imaging and Interventional Radiology Center, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Tran Ngoc Minh
- Department of Pathology, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Dang Duc Thinh
- Department of Pathology, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Nguyen Ngoc Cuong
- Diagnostic Imaging and Interventional Radiology Center, Hanoi Medical University Hospital, Hanoi, Vietnam
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18
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Kristiani E, Natalia S, Ritonga EVF, Djokomuljanto S, Kan EF. Kikuchi-Fujimoto Disease in Pediatrics. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2021. [DOI: 10.1016/j.epsc.2021.101989] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022] Open
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19
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Splenic uptake on FDG PET/CT correlates with Kikuchi-Fujimoto disease severity. Sci Rep 2021; 11:10836. [PMID: 34035381 PMCID: PMC8149816 DOI: 10.1038/s41598-021-90350-z] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/30/2020] [Accepted: 04/26/2021] [Indexed: 12/22/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is usually self-limiting, but prolonged systemic symptoms often result in frequent hospital visits, long admission durations, or missed workdays. We investigated the role of fluorine-18 fluoro-2-deoxy-D-glucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) in assessing KFD severity. We reviewed the records of 31 adult patients with pathologically confirmed KFD who underwent 18F-FDG PET/CT between November 2007 and April 2018 at a tertiary-care referral hospital. Disease severity was assessed using criteria based on clinical manifestations of advanced KFD. Systemic activated lymph nodes and severity of splenic activation were determined using semi-quantitative and volumetric PET/CT parameters. The median of the mean splenic standardized uptake value (SUVmean) was higher in patients with severe KFD than those with mild KFD (2.38 ± 1.18 vs. 1.79 ± 0.99, p = 0.058). Patients with severe KFD had more systemically activated volume and glycolytic activity than those with mild KFD (total lesion glycolysis: 473.5 ± 504.4 vs. 201.6 ± 363.5, p = 0.024). Multivariate logistic regression showed that myalgia (odds ratio [OR] 0.035; 95% confidence interval [CI] 0.001–0.792; p = 0.035), total lymph node SUVmax (cutoff 9.27; OR 24.734; 95% CI 1.323–462.407; p = 0.032), and spleen SUVmean (cutoff 1.79; OR 37.770; 95% CI 1.769–806.583; p = 0.020) were significantly associated with severe KFD. 18F-FDG PET/CT could be useful in assessing KFD severity.
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20
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Nelson ND, Meng W, Rosenfeld AM, Bullman S, Sekhar Pedamallu C, Nomburg JL, Wertheim GB, Paessler ME, Pinkus G, Hornick JL, Meyerson M, Luning Prak ET, Pillai V. Characterization of Plasmacytoid Dendritic Cells, Microbial Sequences, and Identification of a Candidate Public T-Cell Clone in Kikuchi-Fujimoto Disease. Pediatr Dev Pathol 2021; 24:193-205. [PMID: 33530869 DOI: 10.1177/1093526620987961] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
OBJECTIVES Kikuchi-Fujimoto disease (KFD) is a self-limited lymphadenitis of unclear etiology. We aimed to further characterize this disease in pediatric patients, including evaluation of the CD123 immunohistochemical (IHC) staining and investigation of potential immunologic and infectious causes. METHODS Seventeen KFD cases and 12 controls were retrospectively identified, and the histologic and clinical features were evaluated. CD123 IHC staining was quantified by digital image analysis. Next generation sequencing was employed for comparative microbial analysis via RNAseq (5 KFD cases) and to evaluate the immune repertoire (9 KFD cases). RESULTS In cases of lymphadenitis with necrosis, >0.85% CD123+ cells by IHC was found to be six times more likely in cases with a final diagnosis of KFD (sensitivity 75%, specificity 87.5%). RNAseq based comparative microbial analysis did not detect novel or known pathogen sequences in KFD. A shared complementarity determining region 3 (CDR3) sequence and use of the same T-cell receptor beta variable region family was identified in KFD LNs but not controls, and was not identified in available databases. CONCLUSIONS Digital quantification of CD123 IHC can distinguish KFD from other necrotizing lymphadenitides. The presence of a unique shared CDR3 sequence suggests that a shared antigen underlies KFD pathogenesis.
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Affiliation(s)
- Nya D Nelson
- Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.,Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
| | - Wenzhao Meng
- Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Aaron M Rosenfeld
- Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Susan Bullman
- Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, Massachusetts.,Department of Medical Oncology and Center for Cancer Genome Discovery, Dana-Farber Cancer Institute, Boston, Massachusetts.,Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Chandra Sekhar Pedamallu
- Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, Massachusetts.,Department of Medical Oncology and Center for Cancer Genome Discovery, Dana-Farber Cancer Institute, Boston, Massachusetts.,Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Jason L Nomburg
- Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, Massachusetts.,Department of Medical Oncology and Center for Cancer Genome Discovery, Dana-Farber Cancer Institute, Boston, Massachusetts.,Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Gerald B Wertheim
- Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
| | - Michele E Paessler
- Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.,Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
| | - Geraldine Pinkus
- Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Jason L Hornick
- Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Matthew Meyerson
- Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, Massachusetts.,Department of Medical Oncology and Center for Cancer Genome Discovery, Dana-Farber Cancer Institute, Boston, Massachusetts.,Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Eline T Luning Prak
- Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Vinodh Pillai
- Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.,Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
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21
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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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22
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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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23
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Shorkey AK, Laxer RM, Friedman JN. A 10-year-old girl with cervical lymphadenopathy, fever, and cytopenia. Paediatr Child Health 2020; 25:342-344. [DOI: 10.1093/pch/pxz114] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/17/2018] [Accepted: 07/17/2019] [Indexed: 11/14/2022] Open
Affiliation(s)
- Allyson K Shorkey
- Department of Paediatrics, The Hospital for Sick Children, The University of Toronto, Toronto, Ontario
- The Hospital for Sick Children, The University of Toronto, Toronto, Ontario
| | - Ronald M Laxer
- Department of Rheumatology, The Hospital for Sick Children, The University of Toronto, Toronto, Ontario
| | - Jeremy N Friedman
- Department of Paediatrics, The Hospital for Sick Children, The University of Toronto, Toronto, Ontario
- The Hospital for Sick Children, The University of Toronto, Toronto, Ontario
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24
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Cannon L, Wang E, Becker M. Diagnosis of Kikuchi-Fujimoto disease in an 11-year-old girl with fever and sickle cell disease. BMJ Case Rep 2020; 13:13/8/e234302. [PMID: 32843398 DOI: 10.1136/bcr-2020-234302] [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: 11/03/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare lymphohistiocytic disorder which can cause prolonged fever and other systemic B symptoms including diffuse lymphadenopathy. Given its clinical presentation, there is often initial concern for lymphoma and diagnosis requires lymph node biopsy. It most frequently affects young women of Asian descent; it is less commonly encountered in paediatric patients. KFD is typically a benign, self-limited process, however, there is an association with development of systemic lupus erythematosus. Given its rarity, it remains unclear if KFD is associated with other chronic conditions. Here we present the third case of KFD occurring in a paediatric patient with sickle cell disease.
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Affiliation(s)
- Laura Cannon
- Division of Pediatric Rheumatology, Department of Pediatrics, Duke Children's Hospital and Health Center, Durham, North Carolina, USA
| | - Endi Wang
- Department of Pathology, Duke University Hospital, Durham, North Carolina, USA
| | - Mara Becker
- Division of Pediatric Rheumatology, Department of Pediatrics, Duke Children's Hospital and Health Center, Durham, North Carolina, USA
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25
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Selvanathan SN, Suhumaran S, Sahu VK, Chong CY, Tan NWH, Thoon KC. Kikuchi-Fujimoto disease in children. J Paediatr Child Health 2020; 56:389-393. [PMID: 31576642 DOI: 10.1111/jpc.14628] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/18/2019] [Revised: 08/27/2019] [Accepted: 09/01/2019] [Indexed: 02/02/2023]
Abstract
AIM Kikuchi-Fujimoto disease (KFD) is an important cause of lymphadenitis in children. The primary aim of this study was to investigate the clinical characteristics of children with KFD and to assess the recurrence of this disease. METHODS This is a retrospective study of patients younger than 18 years old, who were diagnosed with KFD from January 2000 to September 2017 at KK Women's and Children's Hospital. Records of children with a histological diagnosis of KFD from a lymph node biopsy were obtained from the Department of Pathology. Case notes and electronic medical records of the patients were reviewed. Data collected included patient characteristics, symptoms, clinical and laboratory findings, treatment and follow-up. RESULTS A total of 98 patients were identified. There were 52 boys and 46 girls with a median age of 11.2 years old. Recurrence occurred in 12 (12.2%) patients. One patient developed systemic lupus erythematosus 10 years after diagnosis of KFD. Recurrent cases were more likely to be managed as an inpatient and have fever at presentation of their first episode of KFD. CONCLUSION In our study, KFD in children had a higher prevalence among boys, and had a recurrence rate of 12.2%, with 1% of patients developing systemic lupus erythematosus. We recommend that patients be followed up for recurrence and advised to monitor for symptoms of recurrence.
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Affiliation(s)
- Shoba N Selvanathan
- Department of Paediatric Medicine, KK Women's and Children's Hospital, Singapore
| | - Sharline Suhumaran
- Department of Child Development, KK Women's and Children's Hospital, Singapore
| | - Vinay K Sahu
- Department of Paediatric Medicine, KK Women's and Children's Hospital, Singapore.,Duke-NUS Graduate School of Medicine, National University of Singapore, Singapore.,Yong Loo Lin School of Medicine, National University of Singapore, Singapore.,Lee Kong Chian School of Medicine, National Technological University, Singapore
| | - Chia Yin Chong
- Duke-NUS Graduate School of Medicine, National University of Singapore, Singapore.,Yong Loo Lin School of Medicine, National University of Singapore, Singapore.,Lee Kong Chian School of Medicine, National Technological University, Singapore.,Infectious Disease Service, Department of Paediatrics, KK Women's and Children's Hospital, Singapore
| | - Natalie Woon Hui Tan
- Duke-NUS Graduate School of Medicine, National University of Singapore, Singapore.,Yong Loo Lin School of Medicine, National University of Singapore, Singapore.,Lee Kong Chian School of Medicine, National Technological University, Singapore.,Infectious Disease Service, Department of Paediatrics, KK Women's and Children's Hospital, Singapore
| | - Koh Cheng Thoon
- Yong Loo Lin School of Medicine, National University of Singapore, Singapore.,Lee Kong Chian School of Medicine, National Technological University, Singapore.,Infectious Disease Service, Department of Paediatrics, KK Women's and Children's Hospital, Singapore
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26
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Case Report of Kikuchi-Fujimoto Disease from Sub-Saharan Africa: An Important Mimic of Tuberculous Lymphadenitis. Case Rep Med 2020; 2020:4385286. [PMID: 31969905 PMCID: PMC6969645 DOI: 10.1155/2020/4385286] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/27/2019] [Revised: 10/08/2019] [Accepted: 12/19/2019] [Indexed: 11/20/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare form of painful lymphadenopathy, usually cervical, which is more common in Southeast Asia and rarely reported from Africa. Symptoms are usually nonspecific (fever, night sweats, etc.), and can mimic more common diseases such as tuberculosis (TB) in endemic areas. We report a case of a 29-year-old black African woman who was admitted with headache, neck pain, fever, and lymphadenopathy. She was found to have aseptic meningitis, eventually attributed to TB based on cervical node biopsy, although further histology suggested KFD. Blood tests for systemic lupus erythematosus (SLE) were negative; she had already been commenced on anti-TB treatment and had responded well and so was continued with this therapy. She was also later diagnosed with Hashimoto's thyroiditis 3 months after her diagnosis of KFD. Five months after stopping TB treatment, she was readmitted with the same symptoms and associated painless lymphadenopathy. Repeat biopsy was morphologically similar to that of 2017, and repeat evaluation confirmed SLE. She has since been managed by a rheumatologist and continues to do well.
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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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Altinel Açoğlu E, Sari E, Şahin G, Oğuz MM, Akçaboy M, Zorlu P, Senel S. Kikuchi-Fujimoto disease triggered by Salmonella enteritidis in a child with concurrent auto-immune thyroiditis and papilloedema. Paediatr Int Child Health 2018; 38:298-301. [PMID: 29307273 DOI: 10.1080/20469047.2017.1420523] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
Kikuchi-Fujimoto disease (KFD) is a histiocytic necrotising lymphadenitis characterised by painful cervical lymphadenopathy, fever, malaise and weight loss. Infections, auto-immune pathogenesis and a genetic association have been implicated. A 12-year-old boy presented with a 1-month history of fever, abdominal pain, constipation and weight loss, and a painful lymph node was detected in the right axilla. Chest CT demonstrated multiple lymph nodes, especially in the left mediastinum. Salmonella enteritidis group D was detected in a blood culture and he was treated with ceftriaxone, followed by meropenem. An axillary lymph node biopsy demonstrated necrotising histiocytic lymphadenitis and KFD was diagnosed. He was discharged 35 days after admission. He was re-admitted 3 weeks later with recurrence of symptoms and headache and was found to have papilloedema of the left eye and auto-immune thyroiditis. Intravenous immunoglobulin (IVIG) 400 mg/kg/day was administered for 5 days. The fever and papilloedema slowly resolved and, subsequently, the thyroiditis, and he has remained well on follow-up. This is the first report of an association of S. enteritidis infection and papilloedema with KFD. IVIG may be required in prolonged or recurrent cases and in those with an auto-immune association.
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Affiliation(s)
- Esma Altinel Açoğlu
- a Departments of Pediatrics , Dr Sami Ulus Maternity and Children's Health and Diseases Training and Research Hospital , Ankara , Turkey
| | - Eyup Sari
- a Departments of Pediatrics , Dr Sami Ulus Maternity and Children's Health and Diseases Training and Research Hospital , Ankara , Turkey
| | - Gürses Şahin
- b Departments of Pediatric Oncology , Dr Sami Ulus Maternity and Children's Health and Diseases Training and Research Hospital , Ankara , Turkey
| | - Melahat Melek Oğuz
- a Departments of Pediatrics , Dr Sami Ulus Maternity and Children's Health and Diseases Training and Research Hospital , Ankara , Turkey
| | - Meltem Akçaboy
- a Departments of Pediatrics , Dr Sami Ulus Maternity and Children's Health and Diseases Training and Research Hospital , Ankara , Turkey
| | - Pelin Zorlu
- a Departments of Pediatrics , Dr Sami Ulus Maternity and Children's Health and Diseases Training and Research Hospital , Ankara , Turkey
| | - Saliha Senel
- a Departments of Pediatrics , Dr Sami Ulus Maternity and Children's Health and Diseases Training and Research Hospital , Ankara , Turkey
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Lee EJ, Lee HS, Park JE, Hwang JS. Association Kikuchi disease with Hashimoto thyroiditis: a case report and literature review. Ann Pediatr Endocrinol Metab 2018; 23:99-102. [PMID: 29969882 PMCID: PMC6057018 DOI: 10.6065/apem.2018.23.2.99] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/17/2017] [Accepted: 11/10/2017] [Indexed: 11/20/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disease characterized by fever and lymphadenopathy. The etiology of KFD is unknown, but an autoimmune cause has been suggested. Hashimoto thyroiditis is the most common autoimmune thyroid disorder in children and is known to be associated with other autoimmune diseases. Only a few cases of Hashimoto thyroiditis associated with KFD have been documented. We report a case of a 16-year-old girl who was first diagnosed with KFD and developed Hashimoto thyroiditis 2 years and 6 months later during her follow-up period. Physicians of patients with KFD should consider the possibility of autoimmune diseases like Hashimoto's thyroiditis.
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Affiliation(s)
| | - Hae Sang Lee
- Address for correspondence: Hae Sang Lee, MD, PhD Department of Pediatrics, Ajou University School of Medicine, 206 World cup-ro, Yeongtong-gu, Suwon 16499, Korea Tel: +82-31-219-4454 Fax: +82-31-219-5169 E-mail:
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Wheaton L, Punnett A, Baker JM. A 17-year-old girl with fever and lymphadenopathy. Paediatr Child Health 2018; 23:3-5. [DOI: 10.1093/pch/pxx171] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
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Affiliation(s)
- Dionna Mathews
- 1 Our Lady of the Lake Children's Hospital, Baton Rouge, LA, USA
| | - Pamela McMahon
- 1 Our Lady of the Lake Children's Hospital, Baton Rouge, LA, USA
| | - Michael Bolton
- 1 Our Lady of the Lake Children's Hospital, Baton Rouge, LA, USA
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Tordjman M, Sommet J, Rega A, Benkerrou M, Berrebi D, Peuchmaur M. [An unexplained fever]. Ann Pathol 2017; 37:495-498. [PMID: 29153389 DOI: 10.1016/j.annpat.2017.10.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2016] [Revised: 07/10/2017] [Accepted: 10/10/2017] [Indexed: 11/18/2022]
Affiliation(s)
- Mickael Tordjman
- Service de pathologie, hôpital universitaire Robert-Debré, AP-HP, 48, boulevard Sérurier, 75019 Paris, France.
| | - Julie Sommet
- Service de pédiatrie générale, hôpital Robert-Debré, AP-HP, 75019 Paris, France; Université Diderot Paris 7 - SPC, 16, rue Huchard, 75018 Paris, France
| | - Adelaide Rega
- Service d'imagerie médicale, hôpital Robert-Debré, AP-HP, 75019 Paris, France; Université Diderot Paris 7 - SPC, 16, rue Huchard, 75018 Paris, France
| | - Malika Benkerrou
- Service d'hématologie, centre de drépanocytose, hôpital Robert-Debré, AP-HP, 75019 Paris, France
| | - Dominique Berrebi
- Service de pathologie, hôpital universitaire Robert-Debré, AP-HP, 48, boulevard Sérurier, 75019 Paris, France; Université Diderot Paris 7 - SPC, 16, rue Huchard, 75018 Paris, France
| | - Michel Peuchmaur
- Service de pathologie, hôpital universitaire Robert-Debré, AP-HP, 48, boulevard Sérurier, 75019 Paris, France; Université Diderot Paris 7 - SPC, 16, rue Huchard, 75018 Paris, France
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Lin YC, Huang HH, Nong BR, Liu PY, Chen YY, Huang YF, Chiou YH, Lee HS. Pediatric Kikuchi-Fujimoto disease: A clinicopathologic study and the therapeutic effects of hydroxychloroquine. JOURNAL OF MICROBIOLOGY, IMMUNOLOGY, AND INFECTION = WEI MIAN YU GAN RAN ZA ZHI 2017; 52:395-401. [PMID: 29050748 DOI: 10.1016/j.jmii.2017.08.023] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/23/2017] [Revised: 08/07/2017] [Accepted: 08/31/2017] [Indexed: 12/29/2022]
Abstract
BACKGROUND To investigate the clinical features of Kikuchi-Fujimoto disease (KFD) in children, and place an emphasis on the therapeutic effects of hydroxychloroquine as monotherapy. METHODS We retrospectively reviewed the medical records of all children diagnosed with KFD during the period January 1992 to September 2016 at a tertiary medical center in Taiwan. RESULTS 40 patients were histopathologically confirmed as KFD, and the mean age of the patients was 13.9 ± 3.1 years. The male to female ratio was 1:1. The lymph node involvements were often cervical (95%) with features of unilateral predisposition (75%), polyadenopathy (84.4%) and tenderness (56.3%). Fever, cough, rhinorrhea, and tonsillitis were other common presentations. Laboratory findings included leukopenia (56.5%), monocytosis (63.6%), with positive results of EB-VCA IgG (88.9%), EB-VCA IgM (22.2%), EBEA IgG (22.2%) and EBNA IgG (88.9%). The univariate analyses of prolonged fever with lymphopenia, monocytosis, thrombocytopenia and necrotizing type in histopathology were disclosed as statistically significant (P < 0.05). Corticosteroids and hydroxychloroquine were administered in 15.6% of patients respectively, along with symptomatic treatments for the rest. Recurrence occurred in 13.0% of patients without corticosteroids or hydroxychloroquine treatment. There were neither recurrences nor relevant major adverse effects in all the five KFD cases treated with hydroxychloroquine. CONCLUSION KFD should be suspected in children with febrile cervical lymphadenopathy, especially when concomitant with leukopenia and monocytosis. Lymphopenia, monocytosis, thrombocytopenia and necrotizing type in histopathology are reliable predictors for prolonged fever. Hydroxychloroquine may be an alternative choice to corticosteroids for its favorable effects and safety.
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Affiliation(s)
- Yung-Chih Lin
- Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.
| | - Hsiu-Hui Huang
- Department of Pathology and Laboratory, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan
| | - Bao-Ren Nong
- Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan; Department of Pediatrics, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan
| | - Po-Yen Liu
- Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan
| | - Ying-Yao Chen
- Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan
| | - Yung-Feng Huang
- Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan; Department of Pediatrics, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.
| | - Yee-Hsuan Chiou
- Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan
| | - Herng-Sheng Lee
- Department of Pathology and Laboratory, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan
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Maruyama T, Nishihara K, Saio M, Nakasone T, Nimura F, Matayoshi A, Goto T, Yoshimi N, Arasaki A. Kikuchi-Fujimoto disease in the regional lymph nodes with node metastasis in a patient with tongue cancer: A case report and literature review. Oncol Lett 2017; 14:257-263. [PMID: 28693162 PMCID: PMC5494837 DOI: 10.3892/ol.2017.6139] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/27/2016] [Accepted: 03/09/2017] [Indexed: 02/05/2023] Open
Abstract
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare self-limiting disorder typically affecting the cervical lymph nodes (LNs), which is often misdiagnosed as other LN-associated diseases. KFD frequently presents with necrotic lesions and recurrences, which are also features of metastatic LNs. Clinicians may thus suspect LN metastasis when they encounter ipsilateral cervical lymphadenopathy in a patient with head and neck cancer. The present study reports the case of a 48-year-old man with tongue cancer and KFD affecting the right edge of his tongue and ipsilateral cervical LNs. LN metastasis was initially suspected, but pathological examination of the dissected LNs revealed one necrotic metastatic lesion and two necrotic KFD lesions. Ipsilateral cervical lymphadenopathy recurred 6 years after the initial surgery, and it was not possible to differentiate clinically between a second primary tumor and recurrent KFD prior to treatment. To the best of our knowledge, this is the first reported case of simultaneous tongue cancer, regional LN metastasis and KFD. This highlights the requirement to consider KFD in the event of LNs with necrotic lesions but no cancerous cells. A combination of clinical and pathological approaches may aid in the diagnosis of KFD, in addition to ruling out LN metastasis in initial and recurrent lymphadenopathies. The present study indicate that a diagnosis of KFD should be considered in patients with head and neck cancer that exhibit necrotic LNs lacking cancerous cells. This is important, as misdiagnosis of KFD as LN metastasis may lead to unnecessary adjuvant therapy.
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Affiliation(s)
- Tessho Maruyama
- Department of Oral and Maxillofacial Functional Rehabilitation, Graduate School of Medicine, University of the Ryukyus, Nishihara, Okinawa 903-0215, Japan
- Department of Oral and Maxillofacial Surgery, Ryukyu University Hospital, Nishihara, Okinawa 903-0215, Japan
| | - Kazuhide Nishihara
- Department of Oral and Maxillofacial Functional Rehabilitation, Graduate School of Medicine, University of the Ryukyus, Nishihara, Okinawa 903-0215, Japan
- Department of Oral and Maxillofacial Surgery, Ryukyu University Hospital, Nishihara, Okinawa 903-0215, Japan
| | - Masanao Saio
- Department of Pathology, Ryukyu University Hospital, Nishihara, Okinawa 903-0215, Japan
- Department of Pathology and Oncology, Graduate School of Medicine, University of the Ryukyus, Nishihara, Okinawa 903-0215, Japan
| | - Toshiyuki Nakasone
- Department of Oral and Maxillofacial Surgery, Ryukyu University Hospital, Nishihara, Okinawa 903-0215, Japan
| | - Fumikazu Nimura
- Department of Oral and Maxillofacial Functional Rehabilitation, Graduate School of Medicine, University of the Ryukyus, Nishihara, Okinawa 903-0215, Japan
| | - Akira Matayoshi
- Department of Oral and Maxillofacial Surgery, Ryukyu University Hospital, Nishihara, Okinawa 903-0215, Japan
| | - Takahiro Goto
- Department of Oral and Maxillofacial Surgery, Ryukyu University Hospital, Nishihara, Okinawa 903-0215, Japan
| | - Naoki Yoshimi
- Department of Pathology, Ryukyu University Hospital, Nishihara, Okinawa 903-0215, Japan
| | - Akira Arasaki
- Department of Oral and Maxillofacial Functional Rehabilitation, Graduate School of Medicine, University of the Ryukyus, Nishihara, Okinawa 903-0215, Japan
- Department of Oral and Maxillofacial Surgery, Ryukyu University Hospital, Nishihara, Okinawa 903-0215, Japan
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Kikuchi Disease Causing Fever of Unknown Origin and Generalized Lymphadenopathy. Arch Rheumatol 2017; 32:271-272. [PMID: 30375539 DOI: 10.5606/archrheumatol.2017.6291] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/22/2016] [Accepted: 12/05/2016] [Indexed: 11/21/2022] Open
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Escudier A, Courbage S, Meignin V, Abbou S, Sauvion S, Houlier M, Galerne A, Gaudelus J, de Pontual L, Simonin M. [Kikuchi-Fujimoto disease mimicking malignant lymphoma in adolescents]. Arch Pediatr 2017; 24:471-475. [PMID: 28365190 DOI: 10.1016/j.arcped.2017.02.027] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/13/2016] [Revised: 11/05/2016] [Accepted: 02/21/2017] [Indexed: 10/19/2022]
Abstract
Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a rare cause of lymphadenopathy in children. This benign disease can mimic lymphoma and misleads doctors. It was first described in Asia, where it occurred especially in young women. Recent publications show that it can also affect teenagers and young adults in Caucasian populations. The pathophysiology remains unknown. Three hypotheses have been raised for this disease: the role of viruses (in particular HHV-8), genetic predisposition (two alleles in HLA class II genes were found more frequently in patients with Kikuchi disease), and an autoimmune cause because of the correlation with lupus erythematosus. Few cases have been reported in Europe so far. In this article, we report three cases of Kikuchi disease observed in less than 2 months in a single hospital in France. All three patients were teenagers who presented with lymphadenopathy, either isolated or combined with fever, weakness, and weight loss. In all of them, the hypermetabolic activity of the lymph node on the PET scanner misled us to suspect lymphoma. The diagnosis of Kikuchi disease was finally made, for all patients, after 2 weeks in the hospital based on lymph node biopsy. Based on this report, we highlight that early biopsy in presence of lymphadenopathy can avoid unnecessary extensive investigations. Moreover, in this rare disease, it is very surprising to come across three cases that are not family-related, in such a short period of time. This strengthens the hypothesis of the possible implication of an environmental factor in the pathophysiology of Kikuchi disease.
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Affiliation(s)
- A Escudier
- Service de pédiatrie générale, hôpital Jean-Verdier, hôpitaux universitaires Paris-Seine-Saint-Denis, AP-HP, avenue du 14-Juillet, 93143 Bondy cedex, France
| | - S Courbage
- Service de pédiatrie générale, hôpital Jean-Verdier, hôpitaux universitaires Paris-Seine-Saint-Denis, AP-HP, avenue du 14-Juillet, 93143 Bondy cedex, France
| | - V Meignin
- Service d'anatomopathologie, hôpital Saint-Louis, Assistance publique-Hôpitaux de Paris, 1, avenue Claude-Vellefaux, 75475 Paris cedex 10, France
| | - S Abbou
- Département de pédiatrie, institut Gustave-Roussy, 39, rue Camille-Desmoulins, 94805 Villejuif cedex, France
| | - S Sauvion
- Service de pédiatrie générale, hôpital Jean-Verdier, hôpitaux universitaires Paris-Seine-Saint-Denis, AP-HP, avenue du 14-Juillet, 93143 Bondy cedex, France
| | - M Houlier
- Service de pédiatrie générale, hôpital Jean-Verdier, hôpitaux universitaires Paris-Seine-Saint-Denis, AP-HP, avenue du 14-Juillet, 93143 Bondy cedex, France
| | - A Galerne
- Service de pédiatrie générale, hôpital Jean-Verdier, hôpitaux universitaires Paris-Seine-Saint-Denis, AP-HP, avenue du 14-Juillet, 93143 Bondy cedex, France
| | - J Gaudelus
- Service de pédiatrie générale, hôpital Jean-Verdier, hôpitaux universitaires Paris-Seine-Saint-Denis, AP-HP, avenue du 14-Juillet, 93143 Bondy cedex, France
| | - L de Pontual
- Service de pédiatrie générale, hôpital Jean-Verdier, hôpitaux universitaires Paris-Seine-Saint-Denis, AP-HP, avenue du 14-Juillet, 93143 Bondy cedex, France
| | - M Simonin
- Service de pédiatrie générale, hôpital Jean-Verdier, hôpitaux universitaires Paris-Seine-Saint-Denis, AP-HP, avenue du 14-Juillet, 93143 Bondy cedex, France; Département de pédiatrie, institut Gustave-Roussy, 39, rue Camille-Desmoulins, 94805 Villejuif cedex, France.
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Deaver D, Horna P, Cualing H, Sokol L. Pathogenesis, diagnosis, and management of Kikuchi-Fujimoto disease. Cancer Control 2015; 21:313-21. [PMID: 25310212 DOI: 10.1177/107327481402100407] [Citation(s) in RCA: 69] [Impact Index Per Article: 6.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
BACKGROUND Kikuchi-Fujimoto disease (KFD) is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. This disease is misdiagnosed as malignant lymphoma in up to one-third of cases and is associated with the development of systemic lupus erythematosus (SLE). METHODS The medical literature between the years 1972 and 2014 was searched for KFD, and the data were collected and analyzed regarding the epidemiology, clinical presentations, diagnosis, management, and suggested diagnostic and treatment algorithms. RESULTS Although KFD has been reported in other ethnic groups and geographical areas, it is more frequently diagnosed in young women of Asian descent. Patients with the disease typically present with rapidly evolving tender cervical lymphadenopathy, night sweats, fevers, and headache. Diagnosis is based on histopathological examination. Excisional lymph node biopsy is essential for a correct diagnosis. Apoptotic coagulation necrosis with karyorrhectic debris and the proliferation of histiocytes, plasmacytoid dendritic cells, and CD8(+) T cells in the absence of neutrophils are characteristic cytomorphology features. Interface dermatitis at the onset of KFD may be a marker for the subsequent evolution of SLE. The natural course of the disease is typically benign. Short courses of steroids, nonsteroidal anti-inflammatory drugs, or hydroxychloroquine can be administered to patients with more severe symptoms. CONCLUSIONS Although KFD was described more than 40 years ago, the etiology of this disease remains unsolved. Infectious or autoimmune processes were proposed but have not been definitively confirmed. Clinical presentation with systemic B symptoms and adenopathy may lead to an erroneous diagnosis of malignant lymphoma. The introduction of modern methods into hematopathology, including immunohistochemistry, flow cytometry, and molecular clonality studies, has decreased the probability of misdiagnosis. Until reliable prognostic markers are available, patients with KFD should have continued long-term follow-up care due to their increased risk of SLE.
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Association between Kikuchi-Fujimoto disease and Streptococcus pneumoniae infection. Open Med (Wars) 2014. [DOI: 10.2478/s11536-013-0330-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Abstract
AbstractKikuchi-Fujimoto disease is a rare histiocytic necrotizing lymphadenitis that affects typically young women causing fever and painful laterocervical lymphadenopathy. The etiology is unknown, but several viral infections and autoimmune diseases have been related with the disease. Bacterial infections are less frequent. Diagnosis needs for excisional lymph node biopsy that shows paracortical areas of coagulative necrosis with abundant debris, distortion of the nodal architecture, and a large amount of histiocytes at the margins of the necrotic areas. There is no specific treatment for the disease. We present the case of a young woman with Kikuchi-Fujimoto disease associated with lower respiratory tract infection by Streptococcus pneumoniae and review the literature.
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Yoo IH, Na H, Bae EY, Han SB, Lee SY, Jeong DC, Kang JH. Recurrent lymphadenopathy in children with Kikuchi-Fujimoto disease. Eur J Pediatr 2014; 173:1193-9. [PMID: 24714877 DOI: 10.1007/s00431-014-2306-6] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/16/2013] [Accepted: 03/24/2014] [Indexed: 11/25/2022]
Abstract
UNLABELLED Kikuchi-Fujimoto disease (KFD) is characterized by self-limiting regional lymphadenopathy with prolonged fever. Although the reported recurrence rate of KFD is known to be 3-4 %, this rate appears to be higher in our clinical experience, and rates up to 38.5 % have been previously reported. In this retrospective study, we reviewed medical records of children with pathologically confirmed KFD to investigate the factors associated with recurrent KFD. Enrolled children were divided into two groups according to the recurrence of KFD, and clinical and laboratory factors were compared between the two groups. The recurrence of KFD was determined based not on repeated pathologic confirmation but on the presence of clinical febrile lymphadenopathy. A total of 33 children with KFD, 26 boys (78.8 %) and 7 girls (21.2 %), with a median age of 12 years (9 months to 19 years), were enrolled. Thirty-one children (93.9 %) complained of fever, and most of the children (90.9 %) complained of cervical lymphadenopathy. Neutropenia (<1,500/μL) or lymphopenia (<1,500/μL) was observed in 51.5 %. Lactate dehydrogenase level, erythrocyte sedimentation rate, and C-reactive protein level were elevated in 90.9, 96.9, and 54.5 % of children, respectively. Fourteen children (42.4 %) experienced recurrent KFD, including ten children after biopsy and four children before and after biopsy. In a multivariate analysis, a past history of other systemic illnesses (p = 0.013) and a higher absolute lymphocyte count (p = 0.023) were significantly associated with recurrent KFD. These systemic illnesses were chronic idiopathic thrombocytopenic purpura, autoimmune thyroiditis, nephrotic syndrome, perinatal cytomegalovirus infection, and hemophagocytic lymphohistiocytosis. CONCLUSION Our results suggest that recurrent KFD is more frequent than reported, and recurrent KFD should be considered in children with a history of other systemic illnesses such as immune disorders.
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Affiliation(s)
- In Hyuk Yoo
- Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea,
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