Also known as histiocytic necrotizing lymphadenitis, kfd is usually considered a benign, self. We discuss the clinical features, differential diagnosis, radiography, pathology, and outcome. Report of a fatal adult case with hemophagocytic lymphohistiocytosis, human pathology. Kikuchi fujimoto disease is a selflimited disease that usually resolves within a few months. Fujimoto and colleagues described kikuchi s disease in the same year. Kikuchifujimoto disease rooks textbook of dermatology. Also called histiocytic necrotizing lymphadenitis, kikuchi fujimoto disease initially described in japan and other asian countries. An uncommon presentation of kikuchi fujimoto disease.
Necrosis of lymph node tissue is caused by apoptosis and may be virally induced. Kikuchifujimoto disease is a rare, selflimiting disease of unknown etiology, manifested by lymphadenopathy, fever and less specific symptoms such as. Kikuchifujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence. Mar 18, 2015 the lymph node changes in kawasaki disease mucocutaneous lymph node disease, cat scratch disease and atypical mycobacterial infection are quite different, being characterised by intravascular fibrin thrombi and neutrophils, stellate microabscesses with neutrophils and necrotising stellate granulomatous inflammation respectively. Kikuchifujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a selflimited disease of complex and unclear etiology. Initially, bacterial agents, such as yersinia, bartonella and protozoan toxoplasma were thought to cause the disease, recently atypical mycobacterium szulgi has been isolated from thailand, brucella has also. It is rare in caucasians, normally occurring in those of asian descent. Despite its low incidence, kikuchi fujimoto disease should be considered in patients with persistent lymphadenopathy. Kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among. Overlap between systemic lupus erythematosus and kikuchi fujimoto disease. Kikuchi fujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Clinicopathological correlation sainath k andola1, anshuman sinha2, sangram biradar3 abstract kikuchifujimotos disease is a benign, selflimiting disorder characterized by regional tender lymphadenopathy fever and night sweats. Involvement of intraabdominal lymph nodes has been rarely reported.
Fine needle aspiration usually results in a necrotic aspirate, with absence of epithelioid cells or atypical cells. The lymph node changes in kawasaki disease mucocutaneous lymph node disease, cat scratch disease and atypical mycobacterial infection are quite different, being characterised by intravascular fibrin thrombi and neutrophils, stellate microabscesses with neutrophils and necrotising stellate granulomatous inflammation respectively. Kikuchi s disease, also known as kikuchi fujimoto disease kfd or histiocytic necrotizing lymphadenitis, is a rare disorder with a worldwide distribution, with a higher prevalence among asians. Despite its low incidence, kikuchifujimoto disease should be considered in patients with persistent lymphadenopathy. Less frequent symptoms include weight loss, nausea, vomiting, sore throat. Kikuchi fujimoto disease and systemic lupus erythematosus.
Kikuchifujimoto disease kfd, or histiocytic necrotizing. Mean of kikuchifujimoto disease is 2355 points 65 %. Kikuchi fujimoto disease is a rare, selflimited, histiocytic, necrotizing lymphadenitis first described in japan in 1972. Kikuchifujimoto disease kfd is typically a selflimited, benign illness which presents with fever and lymphadenopathy. Kikuchi fujimoto disease mimicking tuberculous lymphadenitis or lymphoma on fluorine18 fluorodeoxyglucose positron emission tomographycomputed tomography noraini abdul rahim,1 abdul jalil nordin,2 chiara rusconi,3 cristina gabutti,3 erika ravelli,4 claudio rossetti5 1diagnostic imaging department, serdang hospital, selangor, 2centre for diagnostic nuclear imaging, university putra malaysia. First described independently in 1972 by kikuchi and fujimoto et al,1,2 kfd is an.
Jun 01, 2008 this rare entity can be diagnosed only by excisional biopsy on the basis of its characteristic histologic appearance. Fujimoto and colleagues described kikuchis disease in the same year. Kikuchifujimoto disease kfd was first described in japan in 1972. In 1972, kikuchi and fujimoto independently described this entity. Kikuchi fujimoto disease, also known as subacute necrotizing lymphadenitis or subacute necrotizing histiocytosis, is an idiopathic disease characterized usually by cervical lymph node enlargement 80%. To our knowledge, there is no previous study comparing the clinical and laboratory characteristics of patients from different geographical parts of the world. Pathology of kikuchifujimoto disease top ten facts dr. Kikuchifujimoto disease in a 30yearold caucasian female. Kikuchifujimoto disease and systemic lupus erythematosus imcrj. Kfd has a worldwide distribution, and asiatic people have a higher prevalence.
Kikuchifujimoto disease kimura disease leukaemia cutis lymphomatous infiltration of the skin. Histiocytic necrotizing lymphadenitis kikuchifujimoto. It is a rare cause of lymphadenopathy, commonly seen in individuals of asian descent and frequently associated with fever and, sometimes. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchifujimoto disease. Covering all aspects of skin disease from basic science through pathology and epidemiology to clinical practice, the text is recognized for its unparalleled coverage of diagnosis. The differential diagnosis includes lymphoma with necrosis, catscratch disease and lupus lymphadenitis. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchifujimoto disease, is an uncommon cause of lymphadenopathy.
Kikuchi disease kd also known as kikuchifujimoto disease, necrotizing histiocytic lymphadenopathy is an uncommon, benign, enigmatous, selflimiting, rarely fatal, disease of young caucasian females, presenting with firm, tender, unilateral cervical lymphadenopathy. We describe a case of persistent cervical lymphadenopathy secondary to kikuchi fujimoto disease in an 11yearold girl. Also called histiocytic necrotizing lymphadenitis, kikuchifujimoto disease. Patients present with painless cervical lymphadenopathy that may be accompanied by fever. Kikuchi disease genetic and rare diseases information. Initially described in japan, kfd was first reported in 1972 almost simultaneously by kikuchi and by fujimoto et al. Kikuchifujimoto disease previous article addressing invisibility, inferiority, and powerlessness to achieve gains in maternal health for ultrapoor women in february, 20, a 30yearold ugandanborn woman, working in the uk, presented to the accident and emergency department with a 4week history of arthralgia, fevers, and lethargy, and a. We provide 1 original jpeg or tiff and 1 highquality optimized image without our logo. Kikuchis disease, lymphadenitis, lymphoma, systemic lupus erythematosus. Lymphoma may have prominent necrosis, but atypical cells should be present, and plasmacytoid monocytes are lacking.
Kikuchifujimoto disease presenting as pyrexia of unknown origin. It commonly presents with cervical lymphadenitis and fever. Kikuchi disease is an uncommon, benign, rarely fatal, disease, reported independently, almost simultaneously, by kikuchi and fujimoto et al. Kikuchi first described the disease in 1972 in japan. Kikuchifujimoto disease is a unique entity which needs to be identified in patients because inspite of having a sinister presentation of enlarged tender lymph nodes and abundant necrosis on histomorphology, the condition is selflimiting and requires basic symptomatic management. Cervical lymphadenopathy, fever and raised erythrocyte sedimentation rate are usual presenting features of. Kikuchi fujimoto disease is a unique entity which needs to be identified in patients because inspite of having a sinister presentation of enlarged tender lymph nodes and abundant necrosis on histomorphology, the condition is selflimiting and requires basic symptomatic management. May 23, 2006 kikuchi fujimoto disease kfd is an enigmatic, benign and selflimited syndrome characterized by regional lymphadenopathy with tenderness, predominantly in the cervical region, usually accompanied by mild fever and night sweats. Though several viral agents or an autoimmune etiology has been proposed as.
Cervical lymphadenopathy, fever and raised erythrocyte sedimentation rate are usual presenting features of this. Fineneedle aspiration cytology of kikuchi fujimoto disease. Fujimoto and colleagues independently described kikuchi disease in the same year. Pathogenesis, diagnosis, and management of kikuchi. The disease frequently mimics tuberculous lymphadenitis, malign lymphoma, and many other benign and malignant conditions. Statistics of kikuchifujimoto disease 1 people with kikuchifujimoto disease have taken the sf36 survey. Kikuchi fujimoto disease is a benign and selflimited disease with a female predominance affecting preferentially the asian population. We report a case of kfd in a 47 yearold man who presented with weight loss, fever, enlarged lymph nodes and elevated serum levels. Kikuchifujimoto disease is an enigmatic, benign, and selflimited syndrome characterized by. Kikuchifujimoto disease and systemic lupus erythematosus. A higher prevalence of this disease is seen among the asian population. Catscratch disease, caused by bartonella henselae, has a clinical history of cat exposure with an initial skin papule.
Kikuchi fujimotos disease presents as enlarged tender lymphadenopathy accompanied by fever in asian women in the fourth decade. Kikuchifujimoto disease, fdg, petct clin nucl med 2011. To view the images or references, you must click on the links in blue links in green are to journals with free full textno registration this email is sent only to subscribers. Mar 15, 2006 kikuchifujimoto disease kfd was first described in japan in 1972. Initially described in japan and other asian countries. Kikuchi fujimoto disease associated with cryptogenic organizing pneumonia. Kikuchi disease, is a rare pathology of the lymph nodes. Examples of multiuser license include books, book chapters, educational material, and other publications with multiple copies. Kikuchifujimoto disease orphanet journal of rare diseases.
Clinically and microscopically, it may mimic malignant lymphoma and other nonmalignant diseases. Examples of singleuser license include conferences, seminars, presentations, and onetime teaching courses. While most common in southeast asia, where kfd was initially described in 1972 in young women, this lesion has since been reported in patients of virtually any age, gender. These cases can also be accessed by clicking on the case of the week button on the left hand side of our home page at. Lymph nodes show wellcircumscribed necrotizing lesions in paracortical areas. Kikuchifujimoto disease mimicking tuberculous lymphadenitis or lymphoma on fluorine18 fluorodeoxyglucose positron emission tomographycomputed tomography noraini abdul rahim,1 abdul jalil nordin,2 chiara rusconi,3 cristina gabutti,3 erika ravelli,4 claudio rossetti5 1diagnostic imaging department, serdang hospital, selangor, 2centre for diagnostic nuclear imaging, university putra. The cause of kikuchi fujimoto s disease is unknown. Full text kikuchifujimoto disease mimicking tuberculous. Sep 25, 2018 kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchi fujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Ferry, in diagnostic pathology of infectious disease second edition, 2018. Kikuchifujimoto disease, also known as subacute necrotizing lymphadenitis or subacute necrotizing histiocytosis, is an idiopathic disease characterized usually by cervical lymph node enlargement 80%.
It can be confused with lymphoma, adenocarcinoma metastasis and tuberculosis. Kikuchi disease is a selflimiting illness which has symptoms which may overlap with hodgkins lymphoma leading to misdiagnosis in some patients. Kikuchi fujimoto disease also known as histiocytic necrotizing lymphadenitis is an idiopathic benign self limiting condition with a favourable outcome, usually affecting young women. Kikuchi fujimoto disease kfd is a selflimited disease characterized by destruction of lymph node parenchyma.
Enigmatic kikuchifujimoto disease american journal of. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Kikuchifujimoto disease is a rare, selflimited, histiocytic, necrotizing lymphadenitis first described in japan in 1972. Kikuchifujimoto disease radiology reference article. Kikuchifujimoto disease kfd is a benign and selflimited disorder, characterized by regional cervical lymphadenopathy with tenderness, usually accompanied with mild fever and night sweats. Pathogenesis, diagnosis, and management of kikuchifujimoto. Sep 14, 2016 kikuchi disease is a benign noncancerous condition of the lymph nodes.
Few reports have assessed the immunohistologic features of kfd, and most reports employed limited antibody panels that lacked many of novel immunohistochemistry markers currently available. Kikuchi fujimoto disease kfd is a benign and selflimited disorder, characterized by regional cervical lymphadenopathy with tenderness, usually accompanied with mild fever and night sweats. Kikuchifujimoto disease with lymph node, spleen, and. Less common symptoms include weight loss, nausea, vomiting, and sore throat. Kikuchifujimoto disease kfd also known as histiocytic necrotizing. Kikuchi fujimoto disease kfd is a benign, selflimiting disease characterized by histiocytic necrotising lymphadenitis. Although the cause is unknown, infectious and autoimmune etiologies have been proposed for this disease. The clinical features are such that it is often mistaken for infectious. We describe a case of persistent cervical lymphadenopathy secondary to kikuchifujimoto disease in an 11yearold girl. The following cases have burn reported for clinical interest and for the rarity of. Kikuchifujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, was initially described as a distinct histologic pattern of lymphadenitis generally associated with a benign selflimited clinical course. Treatments for kikuchifujimoto disease including drugs, prescription medications, alternative treatments, surgery, and lifestyle changes. Kikuchi fujimoto disease is a rare, selflimiting disease of unknown etiology, manifested by lymphadenopathy, fever and less specific symptoms such as nocturnal diaphoresis, weight loss, headache.
Kikuchifujimoto disease is so named because kikuchi and fujimoto were the first scientists to describe it in japan in 1972. Kikuchi disease is a benign self limiting condition of unknown etiology, although infectious and autoimmune causes have been suggested 46. Although the disease has been reported from all over the world and more so from asia, it is rare. The following cases have burn reported for clinical interest and for the rarity of the reports about this condition in ent literature. Kikuchifujimoto disease forum questions about kikuchifujimoto disease ask a question and get answers from other users. Kikuchi lymphadenitis necrotizing lymphadenitis, kikuchifujimoto disease is a selflimiting disease of unknown etiology usually seen in young women in japan and other asian countries. Antinuclear antibodies, antiphospholipid antibodies, antidsdna, and rheumatoid factor are usually negative, and may help in differentiation from systemic lupus erythematosus. Gordon jk, magro c, lu t, schneider r, chiu a, furman rr, et al. Kikuchifujimoto disease kfd, or histiocytic necrotizing lymphadenitis, was first described in 1972 simultaneously by kikuchi1 and fujimoto and colleagues2 as a lymphadenitis with focal proliferation of histiocytic cells and abundant karyorrhectic debris. Statistics of kikuchi fujimoto disease 1 people with kikuchi fujimoto disease have taken the sf36 survey. Kikuchifujimoto disease kfd, also known as kikuchi disease, is a rare lymphohistiocytic disorder first described in 1972. Kikuchifujimoto disease kfd was first reported in 1972 almost simultaneously by kikuchi and fujimoto and associates.
While most common in southeast asia, where kfd was initially described in 1972 in young women, this lesion has since been. Hnl usually manifests as isolated cervical lymphadenopathy accompanied by fever and night sweats. This book is distributed under the terms of the creative commons attribution 4. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchi fujimoto disease. Kikuchifujimoto disease kfd is an enigmatic, benign and selflimited syndrome characterized by regional lymphadenopathy with tenderness, predominantly in the cervical region, usually accompanied by mild fever and night sweats. Cervical lymphadenopathy secondary to kikuchifujimoto. Covering all aspects of skin disease from basic science through pathology and epidemiology to clinical practice, the text is recognized.
The main symptoms include swollen lymph nodes in the neck, mild fever, and night sweats. Also called histiocytic necrotizing lymphadenitis, kikuchifujimoto disease initially described in japan and other asian countries. Mean of kikuchi fujimoto disease is 2355 points 65 %. Kikuchi fujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, was initially described as a distinct histologic pattern of lymphadenitis generally associated with a benign selflimited clinical course. A benign form of necrotising lymphadenitis of unknown cause, usually affecting young women, with development of lesions in the lymph node characterized by infiltration of the cortex or paracortex by large collections of proliferating histiocytes and complete or, more often. Introduction cervical lymphadenopathy is a common problem in the pediatric population. Kikuchi fujimoto disease kfd is typically a selflimited, benign illness which presents with fever and lymphadenopathy. Kikuchifujimoto disease kfd, necrotizing lymphadenitis is defined pathologically by enlarged lymph nodes with characteristic microscopic findings and nearly always follows a selflimiting benign clinical course. Kikuchifujimoto disease with lymph node, spleen, and liver. This disease is of unknown etiology, but an infectious etiology has been implicated in the. To date only eight cases have been reported from nepal. The clinical features are such that it is often mistaken for.
Total score ranges from 0 to 3,600 being 0 the worst and 3,600 the best. Mar 22, 2014 kikuchi fujimoto disease previous article addressing invisibility, inferiority, and powerlessness to achieve gains in maternal health for ultrapoor women in february, 20, a 30yearold ugandanborn woman, working in the uk, presented to the accident and emergency department with a 4week history of arthralgia, fevers, and lethargy, and a. While the exact cause of this condition is unknown, infectious and autoimmune causes have been suggested. The aetiology is poorly understood, but it appears to be an autoimmune disorder with a possible infectious trigger. It most commonly affects asian adult females younger than 40 years of age.
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