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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: 17] [Impact Index Per Article: 8.5] [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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Zuckerman R, Damiani L, Ayyad HA, Alpert DR. Persistent cervical lymphadenitis in a patient with prior thyroid cancer attributed to Kikuchi-Fujimoto disease. BMJ Case Rep 2018; 2018:bcr-2018-226457. [PMID: 30344150 DOI: 10.1136/bcr-2018-226457] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/09/2023] Open
Abstract
We describe a 50-year-old woman with a history of thyroid cancer who presented with bilateral cervical and submandibular lymphadenopathy, low-grade fevers, and increasing fatigue. The patient underwent lymph node fine-needle aspiration, which showed no evidence of metastatic or lymphoproliferative disease. This procedure was complicated by a parapharyngeal abscess and cellulitis. She was treated unsuccessfully with various courses of antibiotics, but briefly responded to short courses of steroids. As her cervical lymphadenopathy returned, she underwent an excisional lymph node biopsy, which demonstrated caseating granulomatous lymphadenitis. Extensive review of systems, physical examination, laboratory and imaging studies demonstrated no evidence of malignancy, infection or systemic lupus erythematosus . Our patient was clinically diagnosed with Kikuchi-Fujimoto disease and successfully treated with prednisone tapered over 3 months. She remains in clinical remission.
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Affiliation(s)
- Roman Zuckerman
- Rheumatology, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA
| | - Louise Damiani
- Medicine/Oncology, Jersey Shore University Medical Center, Neptune, New Jersey, USA
| | - Hashem A Ayyad
- Pathology, Jersey Shore University Medical Center, Neptune, New Jersey, USA
| | - Deborah R Alpert
- Medicine/Rheumatology, Jersey Shore University Medical Center, Neptune, New Jersey, USA
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Patra A, Bhattacharya SK. SLE Developing in a Follow-Up Patient of Kikuchi's Disease: A Rare Disorder. J Clin Diagn Res 2013; 7:752-3. [PMID: 23730669 DOI: 10.7860/jcdr/2013/5017.2904] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2012] [Accepted: 12/10/2012] [Indexed: 11/24/2022]
Abstract
Kikuchi's disease or the Kikuchi-Fujimoto Disease (KFD) is a very rare, self-limiting, benign form of histiocytic necrotizing lymphadenitis, which is mostly seen in young females. We are presenting a case of Systemic Lupus Erythematosus (SLE) which occurred after 2 years in a patient of Kikuchi's disease during a follow-up examination.
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Affiliation(s)
- Anupam Patra
- Post Graduate Trainee, Respiratory Medicine, Nilratan Sircar Medical College , Kolkata, India
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Kikuchi-fujimoto disease: a case report and literature review. Case Rep Otolaryngol 2012; 2012:497604. [PMID: 22953115 PMCID: PMC3420536 DOI: 10.1155/2012/497604] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/12/2012] [Accepted: 04/09/2012] [Indexed: 12/21/2022] Open
Abstract
Case. 38-year-old lady was referred to the ENT clinic with history of right-sided facial pain, otalgia, and odynophagia. Clinical examination revealed enlarged right-sided lymph nodes in the neck. Further radiological scans showed a mass near the carotid and enlarged level V lymph nodes. Lymphoma was initially suspected. Fine-needle aspiration and excision biopsy were undertaken. Histological analysis later suggested Kikuchi-Fujimoto disease, also known as histiocytic necrotising lymphadenitis. Literature Review. Kikuchi-Fujimoto disease (KFD) was described in 1972 as lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris. KFD, frequently found in East Asian countries, is rare in the UK. No definite aetiology of KFD is known despite autoimmune and infection factors being suggested. The diagnostic hallmark is histological findings from lymph nodes. Malignancy should be excluded. This condition is mainly self-limiting; hence, management is limited to supportive care. Steroid therapy could be used in severe cases. KFD is relatively unknown in the UK and this case report aims to highlight its occurrence in our population.
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Jang SJ, Min Jeon H, Kim D, Yang WI. Myeloperoxidase positive histiocytes in subacute necrotizing lymphadenitis express both CD11c and CD163. ACTA ACUST UNITED AC 2011. [DOI: 10.1111/j.1755-9294.2011.01114.x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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Abstract
Kikuchi’s disease, a rare disorder which usually presents with fever painful lymphadenopathy, rash and arthritis, all of which are close mimickers of infective and immunological disorders. It is essentially a histopathological diagnosis and tests to rule out other connective tissue disorders or infective etiology must be undertaken. We present a series of two cases of kikuchi-fujimoto’s disease presenting primarily with lymphadenopathy and fever in all cases. The first is a case of generalized lymphadenopathy and the second case of kikuchi’s disease with SLE, a rare association. Lymph node excision biopsy and histopathology documented Kikuchi Fujimoto disease in above cases. All the cases improved on follow up and had no residual stigmata.
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Affiliation(s)
- Pankaj Singhania
- Department of Medicine, Medical College and Hospitals, Kolkata, India
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Vassilakopoulos TP, Pangalis GA, Siakantaris MP, Levidou G, Yiakoumis X, Floudas C, Gribabis D, Bouros S, Metaxas I, Dimitriadou EM, Pantazi L, Tsoukala C, Korkolopoulou P, Andreopoulos A, Vaiopoulos G. Kikuchi's lymphadenopathy: a relatively rare but important cause of lymphadenopathy in Greece, potentially associated with the antiphospholipid syndrome. Rheumatol Int 2009; 30:925-32. [PMID: 19693507 DOI: 10.1007/s00296-009-1077-2] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/07/2008] [Accepted: 07/17/2009] [Indexed: 11/30/2022]
Abstract
Kikuchi-Fujimoto disease is a form of reactive lymphadenopathy, which was firstly described in Japan, but is uncommon in the Western world. We retrospectively reviewed the medical records of nine cases of adult or adolescent Kikuchi's disease diagnosed in a single Haematology Unit in Athens, Greece between 1990 and 2006. The median age of the patients was 25 years (14-40) and 8/9 were females. All patients presented with cervical lymphadenopathy sparing the supraclavicular fossa; one had associated axillary lymphadenopathy, seven had fever and two were asymptomatic. The median duration of lymphadenopathy before presentation was 30 days (10-45). Just palpable splenomegaly was recorded in three patients. The median value of the maximal lymph node diameter was 2 cm (1-5) and only 1/9 had nodes >2 cm in their largest diameter. Lymphadenopathy was tender in two patients; hard nodes were observed in three patients. The median leukocyte count was 4.7 x 10(9)/l (2.2-4.9) with a normal differential in 7/9 patients. No infectious agent could be demonstrated. One patient had clinical and laboratory evidence of primary antiphospholipid syndrome (APLS). In conclusion, Kikuchi's disease represents a rare but important diagnostic possibility for patients presenting with lymphadenopathy in Greece and other western countries. In this setting, autoimmune disorders, mainly lupus and APLS, should be considered and excluded by the appropriate laboratory work-up.
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Affiliation(s)
- Theodoros P Vassilakopoulos
- First Department of Internal Medicine and Department of Haematology, National and Kapodistrian University of Athens, Laikon General Hospital, 17 Ag. Thoma street, Goudi, Athens, 11527, Greece
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8
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Osborn M, Aqel N, Levine TS. The fine needle aspiration appearances of Kikuchi’s lymphadenitis. Cytopathology 2009; 20:36-43. [DOI: 10.1111/j.1365-2303.2007.00491.x] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
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Hrycek A, Cieślik P, Szkróbka W, Pajak J. Kikuchi-Fujimoto disease: a case report. Rheumatol Int 2005; 26:179-81. [PMID: 15947977 DOI: 10.1007/s00296-005-0609-7] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/10/2004] [Accepted: 02/03/2005] [Indexed: 11/25/2022]
Abstract
Kikuchi-Fujimoto disease is a rare benign cervical lymphadenopathy, which often affects young adult women. Its etiology and pathogenesis are unknown. We present the case of Kikuchi-Fujimoto disease in the Polish population and analyse the difficulties in differentiating this disease from the systemic lupus erythematosus.
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Affiliation(s)
- Antoni Hrycek
- Department of Internal Medicine and Clinical Pharmacology, Medical University of Silesia, Katowice, ul. Tysiaclecia 86a/34, 40-871, Katowice, Poland.
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Rezai K, Kuchipudi S, Chundi V, Ariga R, Loew J, Sha BE. Kikuchi-Fujimoto disease: hydroxychloroquine as a treatment. Clin Infect Dis 2004; 39:e124-6. [PMID: 15578393 DOI: 10.1086/426144] [Citation(s) in RCA: 58] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/15/2004] [Accepted: 09/01/2004] [Indexed: 02/01/2023] Open
Abstract
We describe a case of recurrent Kikuchi's disease in a South Asian-American man that was treated successfully with chloroquine and on recurrence with hydroxychloroquine. Each treatment led to a very prompt response.
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Affiliation(s)
- Katayoun Rezai
- Section of Infectious Diseases, Department of Medicine, John H. Stroger Hospital of Cook County, Chicago, IL 60612, USA.
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Santana A, Lessa B, Galrão L, Lima I, Santiago M. Kikuchi-Fujimoto's disease associated with systemic lupus erythematosus: case report and review of the literature. Clin Rheumatol 2004; 24:60-3. [PMID: 15517448 DOI: 10.1007/s10067-004-0923-6] [Citation(s) in RCA: 68] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/01/2003] [Accepted: 03/15/2004] [Indexed: 11/26/2022]
Abstract
Kikuchi-Fujimoto's disease (KFD) or histiocytic necrotising lymphadenitis is a benign and self-limited disease, of unknown aetiology, which affects mainly young women. It presents with localised lymphadenopathy, predominantly in the cervical region, accompanied by fever and leukopenia in up to 50% of the cases. KFD has been rarely described in association with systemic lupus erythematosus (SLE), and its diagnosis can precede, postdate or coincide with the diagnosis of SLE. We present a patient with the diagnosis of SLE characterised by arthritis, leukopenia, malar rash, photosensitivity and positive ANA, besides cervical lymphadenopathy whose biopsy was compatible with KFD, which improved after using prednisone. Although the presence of lymphadenopathy is not uncommon in SLE patients, particularly in the phases of disease activity, the concomitance with KFD has rarely been reported in the literature. Its recognition is necessary because one can avoid laborious investigation for infectious and lymphoproliferative diseases.
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Affiliation(s)
- Alex Santana
- Hospital Santa Izabel, Núcleo de Reumatologia da Bahia/Escola Bahiana de Medicina e Saúde Pública (EBMSP), Praça Almeida Couto, 500, CEP 40.000-000 Nazaré, Salvador, Bahia, Brazil
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Chiang YC, Chen RMY, Chao PZ, Yang TH, Lee FP. Pediatric Kikuchi-Fujimoto disease masquerading as a submandibular gland tumor. Int J Pediatr Otorhinolaryngol 2004; 68:971-4. [PMID: 15183591 DOI: 10.1016/j.ijporl.2004.02.006] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/09/2003] [Accepted: 02/10/2004] [Indexed: 11/18/2022]
Abstract
Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a rare disorder that typically affects the cervical lymph nodes. The disease usually occurs in women in their late 20s or early 30s. Reports in the pediatric literature are sparse. Most authors consider Kikuchi-Fujimoto disease as a self-limiting disorder that requires no specific management but long-term follow-up. The clinical features of Kikuchi-Fujimoto disease are easily confused with other less-benign conditions. Thus, an early biopsy is instrumental in making definite diagnosis and preventing unnecessary investigations. We describe a case of Kikuchi-Fujimoto disease in an 8-year-old boy which presenting as a submandibular gland tumor. The case illustrates the clinical features of this unusual condition and emphasizes the potential confusion with other diagnoses.
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Affiliation(s)
- Yuh-Chyun Chiang
- Department of Otolaryngology, Taiwan Adventist Hospital No. 424, Pa Te Road, Section 2, Taipei, Taiwan
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Affiliation(s)
- Vikram K Mahajan
- Department of Dermatology, Indira Gandhi Medical College, Shimla, India
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14
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Hu S, Kuo TT, Hong HS. Lupus lymphadenitis simulating Kikuchi's lymphadenitis in patients with systemic lupus erythematosus: a clinicopathological analysis of six cases and review of the literature. Pathol Int 2003; 53:221-6. [PMID: 12675765 DOI: 10.1046/j.1320-5463.2003.01458.x] [Citation(s) in RCA: 68] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Kikuchi's disease (KD) or Kikuchi's lymphadenitis (KL) is a self-limiting disease mostly affecting the cervical lymph nodes of young individuals. Whether the reported cases of KL associated with systemic lupus erythematosus (SLE) were genuine KL or lupus lymphadenitis (LL) simulating KL in SLE patients is not clear. We analyzed six cases of KD-like lymphadenitis occurring in SLE patients and 12 reported cases to clarify the relationship between KL and SLE. We found that not all cases occurred simultaneously with SLE. Eight cases occurred either before or after SLE. These cases might have true KL independent of SLE with the exception of two cases that occurred after SLE, but the patients still had lupus activity. The 10 cases that coexisted with SLE most likely had LL rather than KL. This was supported by the immunohistochemical finding of sparse cytotoxic T cells in those lymph nodes in contrast to abundant cytotoxic T cells usually seen in a typical KL. We conclude that KL is not related to SLE, and KD-like lymphadenitis coexisting with SLE should be regarded as LL. Pathologists should be aware of the possibility that LL can mimic KL in patients with SLE, especially necrotizing-type KL.
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Affiliation(s)
- Sindy Hu
- Department of Dermatology, Chang Gung University School of Medicine and Chang Gung Memorial Hospital, Kwei San, Tao Yuan, Taiwan
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Ben Ghorbel I, Houman MH, Lamloum M, Khanfir M, Miled M, Kchir N, Bellil K, Sahtout S. [Concomitant association of Kikuchi disease and systemic lupus erythematosus. Case report]. Rev Med Interne 2002; 23:797-9. [PMID: 12378836 DOI: 10.1016/s0248-8663(02)00679-3] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
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Myeloperoxidase expression by histiocytes in Kikuchi's and Kikuchi-like lymphadenopathy. THE AMERICAN JOURNAL OF PATHOLOGY 2001; 159:915-24. [PMID: 11549584 PMCID: PMC1850446 DOI: 10.1016/s0002-9440(10)61767-1] [Citation(s) in RCA: 70] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Abstract
Forty-five examples of Kikuchi's lymphadenitis (KL), 5 Kikuchi-like lupus erythematosus lymphadenopathies, 25 nonnecrotizing lymphadenitidies (5 toxoplasmic, 5 sarcoid-like, 6 dermatopathic, 4 suppurative, 3 tubercular, 2 with sinus histiocytosis), 4 examples of hyaline-vascular Castleman disease (CD), 2 plasmacytoid monocyte tumors (PM-Ts), and 61 accessory cell neoplasms were studied by a panel of antibodies, including the PG-M1 (against a macrophage-restricted CD68 epitope) and a polyclonal anti-myeloperoxidase (MPO). In KL and Kikuchi-like lupus erythematosus lymphadenopathies, 25 to 75% of CD68(+) histiocytes co-expressed MPO. This did not occur in nonnecrotizing lymphadenitidies and accessory cell neoplasms. MPO(+)/CD68(+) elements corresponded to nonphagocytosing mononuclear cells and some crescentic macrophages and phagocytosing histiocytes. Typical PMs were MPO(-)/CD68(+) in all cases, including CD and PM-T. Our observations suggest that in KL and KL-like lymphadenopathies: 1) MPO(+)/CD68(+) blood monocytes might be attracted into tissues because of the lack or paucity of granulocytes and the need of MPO for oxidative processes; 2) PMs are more likely to be involved in the cytotoxic immune reaction than in phagocytic phenomena; 3) the peculiar phenotype of the histiocytic component can be usefully used for the differentiation from malignant lymphoma and PM-T.
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Keogh MA, Williamson RM, Denaro CP. Kikuchi's disease associated with parotidomegaly, thyroiditis and a rash in a young man. AUSTRALIAN AND NEW ZEALAND JOURNAL OF MEDICINE 2000; 30:633-4. [PMID: 11108076 DOI: 10.1111/j.1445-5994.2000.tb00867.x] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/01/2022]
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Lerosey Y, Lecler-Scarcella V, Francois A, Guitrancourt JA. A pseudo-tumoral form of Kikuchi's disease in children: a case report and review of the literature. Int J Pediatr Otorhinolaryngol 1998; 45:1-6. [PMID: 9804013 DOI: 10.1016/s0165-5876(98)00027-5] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
We report one case of a cervical pseudo-tumoral form of histiocytic necrotizing lymphadenitis (Kikuchi Fugimoto disease) occurring in a 13-year-old child. Diagnosis was made only by histology examination of an excision biopsy from one cervical lymph node. Spontaneous complete resolution occurred within 2 months. A review of the clinical and histological features in adults and children is presented. No difference was found between the two populations regarding these features. Differential diagnoses are malignant lymphoma, systematic lupus erythematosus and, in particular, Still's disease in children. The etiology of the disease is also discussed.
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Affiliation(s)
- Y Lerosey
- Department of Head and Neck Surgery, Rouen University Hospital, France
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Affiliation(s)
- A A Al Salloum
- Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi Arabia
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Sire S, Djossou F, Deminière C, Constans J, Ragnaud JM, Aubertin J. [Kikuchi-Fujimoto necrotizing histiocytic lymphadenitis: apropos of 2 cases and review of the literature]. Rev Med Interne 1996; 17:842-5. [PMID: 8976979 DOI: 10.1016/0248-8663(96)82689-0] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
The Kikuchi-Fujimoto's syndrome is an histiocytic necrotizing lymphadenitis which is observed at any ages but preferentially among young adults. The sex ratio is about 4:1 in favour of women. It is clinically characterized by cervical lymph nodes in a context of fever and asthenia. Sometimes, there is transitory leucopenia and an increase of the erythrocyte sedimentation rate. The course of the disease is spontaneously favourable in 1 or 3 months but recurrence is possible. The histology of the lymph node could mimic a malignant lymphoma and the immunohistochemical findings are of a great importance (Ki-M1P or KP1 antibody). The etiology remains unknown but some infectious diseases have been suspected (toxoplamosis, Epstein-Barr virus). Its association with a systemic lupus erythematous had been described and this set the problem of its physiopathology. We report two new cases of Kikuchi-Fujimoto's syndrome which one was attributed to Epstein-Barr virus primo-infection and the other associated with a systemic lupus erythematous.
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Affiliation(s)
- S Sire
- Clinique médicale et des maladies infectieuses, CHU Bordeaux, hôpital Pellegrin, Bordeaux, France
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Bousquet E, Tubéry M, Brousset P, Anzieu B, Dubarry B, Massip P, Duffaut M. [Kikuchi syndrome, Hashimoto thyroiditis and lupus serology. Apropos of a case]. Rev Med Interne 1996; 17:836-8. [PMID: 8976977 DOI: 10.1016/0248-8663(96)82687-7] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
Histiocytic necrotizing lymphadenitis (Kikuchi's disease) is an uncommon disease of the cervical lymph nodes occurring in young women, commonly associated with various auto-immune or infectious diseases. We describe the case of a 17 year-old girl who presented a Kikuchi's lymphadenitis occurring concomitantly with Hashimoto's thyroiditis and lupus serology as anti-nuclear, anti-DNA, anticardiolipid antibodies and hypocomplementemia. The patient was treated with prednisone and hydroxychloroquine. Thirty months after, she is doing well and hydroxychloroquine is continued.
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Affiliation(s)
- E Bousquet
- Service de médecine interne, CHU de Rangueil, Toulouse, France
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Eisner MD, Amory J, Mullaney B, Tierney L, Browner WS. Necrotizing lymphadenitis associated with systemic lupus erythematosus. Semin Arthritis Rheum 1996; 26:477-82. [PMID: 8870115 DOI: 10.1016/s0049-0172(96)80028-x] [Citation(s) in RCA: 64] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
OBJECTIVE Systemic lupus erythematosus (SLE) may have protean manifestations, including necrotizing lymphadenitis. After describing an illustrative case, we discuss the incidence, clinical features, and pathologic findings of SLE-associated necrotizing lymphadenitis. METHODS A case of SLE associated with necrotizing lymphadenitis is reported. The patient's clinical presentation, course, and response to therapy is detailed. The literature on lupus lymphadenitis is reviewed. RESULTS A young man who presented with a febrile illness characterized by multifocal necrotizing lymphadenitis is described. Glomerulonephritis, meningo-encephalitis, pericarditis, and hemolytic anemia evolved. The diagnosis of SLE was based on the clinical features, positive antinuclear antibody (ANA), and characteristic renal biopsy. High dose corticosteroids and cyclophosphamide induced a complete remission. In recent series from the literature the prevalence of lymphadenopathy was 12% to 59% of patients with SLE. The most common nodal groups involved were cervical (43%), mesenteric (21%), axillary (18%), and inguinal (17%). Lymph node pathology was characterized by paracortical foci of necrosis and infiltration by histiocytes, lymphocytes, plasma cells, and immunoblasts. The hematoxylin body, an amorphic aggregate of basophilic material, was pathognomonic of lupus lymphadenitis. The necrotizing lymphadenitis of SLE is pathologically similar to Kikuchi-Fujumoto disease (KFD), a distinctive, self-limited form of necrotizing lymphadenitis. The pathologic and clinical literature support a close link between SLE and KFD. CONCLUSIONS SLE can be complicated by necrotizing lymphadenitis, with distinctive pathologic features. Lupus lymphadenitis and KFD share some common clinical and pathologic features, supporting a relationship between the disorders.
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Affiliation(s)
- M D Eisner
- Department of Medicine, University of California San Francisco, USA
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Gourley I, Bell AL, Biggart D. Kikuchi's disease as a presenting feature of mixed connective tissue disease. Clin Rheumatol 1995; 14:104-7. [PMID: 7743734 DOI: 10.1007/bf02208094] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/26/2023]
Abstract
Necrotising histiocytic lymphadenitis (Kikuchi's disease) is a recognised cause of benign lymphadenopathy. It has been reported in association with both adult Still's disease, systemic lupus erythematosus (SLE), and mixed connective tissue disease (MCTD). We report a case of mixed connective tissue disease (MCTD), in which biopsy of enlarged lymph nodes occurring as a presenting feature showed the characteristic features of Kikuchi's disease. This finding supports the belief that Kikuchi's disease and the autoimmune rheumatic disorders may share a common aetiology.
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Affiliation(s)
- I Gourley
- Department of Rheumatology, Musgrave Park Hospital, Belfast, Northern Ireland, UK
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Abstract
Histiocytic necrotizing lymphadenitis or Kikuchi disease is a rare entity; furthermore, its association with systemic lupus erythematosus (SLE) was only described in 11 patients in seven reports in the world literature. As the disease affects young women and manifests as a systemic illness associated with lymphadenopathy (usually cervical), the importance of continued follow-up to see whether it remains a self-limiting process or evolves into systemic lupus erythematosus should be emphasized. We present the clinical and histopathological data of eight patients with Kikuchi disease diagnosed in one tertiary care centre, two of whom evolved into SLE. White blood count was normal in all except the two patients who evolved into SLE; erythrocyte sedimentation rate was elevated in all patients tested. Kikuchi disease could be misdiagnosed as non-Hodgkin's lymphoma or tuberculous lymphadenitis; furthermore, awareness of its association with SLE is emphasized.
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Affiliation(s)
- K M el-Ramahi
- Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
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Greenberg ML, Cartwright L, McDonald DA. Histiocytic necrotizing lymphadenitis (Kikuchi's disease): cytologic diagnosis by fine-needle biopsy. Diagn Cytopathol 1993; 9:444-7. [PMID: 8261852 DOI: 10.1002/dc.2840090414] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/29/2023]
Abstract
A cytologic diagnosis of histiocytic necrotizing lymphadenitis (Kikuchi's lymphadenitis) was made in a 14-yr-old female with cervical lymphadenopathy, fever, neutropenia, and hepatosplenomegaly. A predominance of reticulum cells, foamy macrophages, and karyorrhectic debris are clues to the diagnosis in the fine-needle biopsy smears. Subsequent histology confirmed the diagnosis of Kikuchi's lymphadenitis. The differential diagnoses are discussed including malignant lymphoma, which was excluded by morphology as well as flow cytometry and polymerase chain reaction (PCR) studies.
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Affiliation(s)
- M L Greenberg
- Department of Cytology, Westmead Hospital, NSW, Australia
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