Antinuclear antibodies, antiphospholipid antibodies, antidsdna, and rheumatoid factor are usually negative, and may help in differentiation from systemic lupus erythematosus. Kikuchi fujimoto disease kfd is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, kikuchi lymphadenitis, kikuchifujimoto disease, and subacute necrotizing lymphadenitis, is of unknown cause. Aseptic meningitis in kikuchi fujimoto disease is described in the literature but is rare. A 40 year old woman developed recurrent kikuchi s disease 12 years after the original episode. Pathology of kikuchifujimoto disease top ten facts dr. Gaman m, vladareanu am, dobrea c, onisai m, marinescu c, voican i, et al. Kikuchi fujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare, benign, and selflimited disease that causes lymphadenopathy and has a characteristic histological appearance. To date only eight cases have been reported from nepal. Kikuchifujimoto disease kfd was first reported in 1972 almost simultaneously by kikuchi and fujimoto and associates. Histiocytic necrotizing lymphadenitis kikuchifujimoto disease. Pathogenesis, diagnosis, and management of kikuchi. A 28yearold pakistani man with previously treated latent tuberculosis tb presented with a 3month history of productive cough, fever, drenching night sweats, anorexia, sore throat and tender left cervical lymphadenopathy. Serology was negative for toxoplasmosis, ebv or catscratch disease.
It was first described in 1972 by kikuchi and fujimoto in japan independently. Cervical lymphadenopathy, fever and raised erythrocyte sedimentation rate are usual presenting features of this. Lupetin, md, and camilla graham, bs we report the computed tomographic appearance of kikuchi fujimoto disease, a selflimited necrotizing cervical lymphadenitis, that can closely mimic the clinical, pathological, and imaging findings of malignant lymphoma. Kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among. Mar 15, 2006 kikuchifujimoto disease kfd was first described in japan in 1972. A challenging case of kikuchifujimoto disease associated.
The disease was described in 1972 by kikuchi 1 and independently by fujimoto et al 2. It most commonly affects adults younger than 40 years of age and of asian descent. Kikuchifujimoto disease associated to the epsteinbarr. 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.
Although ct findings of kikuchi disease kd, or histiocytic necrotizing lymphadenitis, are reported in several case reports, largescale analysis of the disease has not been undertaken. Full text kikuchifujimoto disease mimicking tuberculous. Kikuchi disease, also called kikuchi fujimoto disease or kikuchi histiocytic necrotizing lymphadenitis, was originally described in young women and is a rare, benign condition of unknown cause usually characterized by cervical lymphadenopathy and fever. Here, we describe a case of kfd which initially presented with fever of unknown origin. We studied 32 patients of the surgical pathology service with necrotizing lymphadenitis, diagnosed in the years from 2004 to 2012 to found. Recognition of this condition is crucial, especially because it can easily be mistaken for tuberculosis, lymphoma, or even adenocarcinoma. Kikuchifujimoto disease kfd is a benign and selflimited disorder, characterized by regional cervical lymphadenopathy with tenderness, usually accompanied with mild fever and night sweats. Histiocytic necrotising lymphadenitis kikuchi fujimoto disease in. 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. Enigmatic kikuchifujimoto disease american journal of.
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. American society for clinical pathology academy of clinical. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchi fujimoto disease, is an uncommon cause of lymphadenopathy. 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. The disease frequently mimics tuberculous lymphadenitis, malign lymphoma, and many other benign and malignant conditions. Kikuchi fujimoto disease is a rare, selflimiting disease of unknown etiology, manifested by lymphadenopathy, fever and less specific symptoms such. Anatomic pathology, university of catania, italy ey ords kikuchi fujimoto disease histiocytic necrotizing lymphadenitis cervical adenopathy fever summary kikuchi fujmoto disease kfd, also known as histiocytic nec. Kikuchi fujimoto disease kfd, a rare clinicopathological entity, is a benign and selflimiting disease.
Enigmatic kikuchifujimoto disease american journal of clinical. Kikuchi disease is a selflimiting illness which has symptoms which may overlap with hodgkins lymphoma leading to misdiagnosis in some patients. We report a 25 yr old female who presented with fever, polyarthritis and cervical lymphadenopathy. Ct appearance of kikuchifujimoto disease sciencedirect. Kikuchi fujimoto disease is rare in children, however, it is well documented in the literature. Advertising and corporate services advertising mediakit reprints and eprints sponsored supplements branded books. Home education eatlas histopathology lymph node kikuchifujimoto disease histiocytic necrotizing. Despite its low incidence, kikuchi fujimoto disease should be considered in patients with persistent. 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. The following cases have burn reported for clinical interest and for the rarity of the reports about this condition in ent literature.
Differentiation from tuberculous lymphadenitis based on patterns of nodal necrosis on ct. No evidence of tuberculosis was found on staining, culture, or the polymerase chain reaction. 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. The recurrence affected the same site axilla and occurred after the longest delay so far recorded in a european resident.
Examples of multiuser license include books, book chapters, educational material, and other publications with multiple copies. Kikuchi fujimoto disease find, read and cite all the research you need on researchgate we use cookies to make interactions with our website easy and. May 23, 2006 kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among japanese and other asiatic individuals. While most common in southeast asia, where kfd was initially described in 1972 in young women, this lesion has since been. Pdf kikuchifujimoto disease in a young african american. Kfd has a worldwide distribution, and asiatic people have a higher prevalence. To our knowledge, there is no previous study comparing the clinical and laboratory characteristics of patients from different geographical parts of the world. Less common symptoms include weight loss, nausea, vomiting, and sore throat. Kikuchifujimoto disease kfd was first described in japan in 1972. An atypical presentation of kikuchifujimoto disease. Kikuchi fujimoto disease kfd or histiocytic necrotizing lymphadenitis is a rare disease that is frequently underdiagnosed due to clinical features that are similar to those of nonhodgkin lymphomas, systemic lupus erythematosus sle, or infectious reactive lymphadenopathy. Kikuchi fujimoto disease is a rare, selflimited, histiocytic, necrotizing lymphadenitis first described in japan in 1972. Kikuchi fujimoto disease is a benign and selflimited disease with a female predominance affecting preferentially the asian population. Histiocytic necrotizing lymphadenitis kikuchis disease.
Oct 15, 2019 kikuchi fujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare, benign, and selflimited disease of unknown etiology that causes lymphadenopathy and has a characteristic. Kikuchi fujimoto disease or histiocytic necrotizing lymphadenitis is a rare form of lymphadenitis. Kikuchi fujimoto disease is a rare benign, condition of necrotising histiocytic lymphadenitis. A young boy presented with a rash, fever, and cervical lymphadenopathy, originally thought to be caused by tuberculosis.
Kikuchi fujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is a specific and selflimited disease. The main symptoms include swollen lymph nodes in the neck, mild fever, and night sweats. While the exact cause of this condition is unknown, infectious and autoimmune causes have been suggested. Recurrence of kikuchis lymphadenitis after 12 years. Soon after the first report, cases were reported from other parts of the world 9. Kikuchi disease is a benign noncancerous condition of the lymph nodes. Here, we present a case of a 66yearold italian woman who was extensively examined for right unilateral. We report a young caucasian female diagnosed with kfd with. Histiocytic necrotizing lymphadenitis kikuchifujimoto. Histopathology of the involved lymph nodes differentiates kikuchi disease from several more. Cervical lymphadenopathy, fever and raised erythrocyte sedimentation rate are usual.
Kikuchi fujimoto disease kfd was described in 1972 as lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris. It is also known as histiocytic necrotising lymphadenitis, abbreviated hnl, and kikuchi fujimoto disease. Care must be taken to avoid misdiagnosis of kikuchi s disease as lymphoma. Kikuchifujimoto disease kfd may have an autoimmune etiology and some cases have been associated with silicone breast. A clinicopathologic study of 79 cases with an analysis of histologic subtypes, immunohistology, and dna ploidy. Kikuchifujimoto disease is a benign and selflimited disease with a female. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchifujimoto disease, is an uncommon cause of lymphadenopathy. An uncommon presentation of kikuchifujimoto disease as.
Kikuchi fujimoto disease in patient with systemic phacomatosis pigmentovascularis. Since then, kfd has been reported worldwide in a variety of ethnic backgrounds. Its diagnosis can be challenging, as the disease must be differentiated from lymphoma and systemic lupus erythematosus. Kikuchis disease nord national organization for rare. Kikuchifujimoto disease associated to the epsteinbarr virus. It is a rare, self limiting, benign form of histiocytic necrotizing lymphadenitis, which can be mistaken for tuberculosis, lymphoma or systemic lupus. Kikuchi fujimoto disease was diagnosed after cervical lymph node biopsy. It most commonly affects asian adult females younger than 40 years of age.
Kikuchi s disease histiocytic necrotizing lymphadenitis. Kikuchifujimoto disease is an extremely rare disease known to have a worldwide. We characterized the clinical and ct findings in a large group of patients with kd. 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%. The clinical, histopathological and immunohistochemical features appear to point to a viral etiology, a hypothesis that still has not been proven. Awareness of this disorder helps prevent misdiagnosis and inappropriate treatment.
Kikuchifujimoto disease kfd is a rare, selflimiting disorder that typically affects the cervical lymph nodes. Also known as histiocytic necrotizing lymphadenitis, kfd is usually considered a benign, self. The illness in may 1999 was similar to the present one and so this case represents a case of recurrent kikuchi fujimoto. Hnl usually manifests as isolated cervical lymphadenopathy accompanied by fever and night sweats.
Mediastinal lymphadenopathy without cervical lymphadenopathy in a case of kikuchi fujimoto disease. We provide 1 original jpeg or tiff and 1 highquality optimized image without our logo. Pathogenesis, diagnosis, and management of kikuchifujimoto. Pdf on jan 1, 20, lida radfar and others published.
Lymph node biopsy revealed the diagnosis of kikuchi fujimoto disease. The objective of the present article is to emphasize the clinicopathological characteristics of this disease that has been associated to the epsteinbarr virus and to. It commonly presents with cervical lymphadenitis and fever. It is also known as histiocytic necrotising lymphadenitis, abbreviated hnl, and kikuchifujimoto disease. Successful treatment of severe kikuchis disease with. Kikuchi fujimoto disease masquerading as tuberculosis. This disease is of unknown etiology, but an infectious etiology has. Although the cause is unknown, infectious and autoimmune etiologies have been proposed for this disease. Kikuchi fujimoto disease associated to the epsteinbarr virus. Histiocytic necrotizing lymphadenitis hnl is a rare benign disorder. To determine the clinicopathologic significance of kikuchifujimoto.
Kikuchi disease is characterized histopathologically by. Now customize the name of a clipboard to store your clips. Patients can present with nonspecific cutaneous lesions. Kikuchifujimoto disease and systemic lupus erythematosus imcrj. Sep 14, 2016 kikuchi disease is a benign noncancerous condition of the lymph nodes. He presented with fevers, weight loss and tender cervical lymph nodes. Kikuchi disease, is a rare pathology of the lymph nodes.
Kikuchifujimoto disease kfd, also known as kikuchi disease, is a rare lymphohistiocytic disorder first described in 1972. Kikuchifujimoto disease radiology reference article. This disorder, a lso called histiocytic necrotizing lymphadenitis and kikuchis disease, was initially described in japan and other asian countries, but now is found worldwide. Kikuchi s disease is an idiopathic illness characterized by a selflimiting lymphadenitis that normally resolves over subsequent weeks or months without specific treatment. This disease is misdiagnosed as malignant lymphoma in up to onethird of cases and is as sociated with the development of systemic lupus erythematosus sle. Textbook of autoantibodies, 2nd edition, elsevier, am. After graduating he trained in internal medicine for two years before moving to work in the pathology department at the school of medicine, kyushu university, in fukuoka city, where he trained in general histopathology and haematopathology. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchifujimoto disease. Clinicopathological correlation sainath k andola1, anshuman sinha2, sangram biradar3 abstract kikuchi fujimoto s disease is a benign, selflimiting disorder characterized by regional tender lymphadenopathy fever and night sweats.
Haemophagocytic syndrome and histiocytic necrotising. Kikuchi fujimoto disease also known as histiocytic necrotizing lymphadenitis is an idiopathic benign self limiting condition with a favourable outcome, usually affecting young women. Less frequent symptoms include weight loss, nausea, vomiting, sore throat. Kikuchifujimoto diseaselike inflammatory reaction in a silicone. Kikuchi fujimoto disease kfd was first described in japan in 1972 almost simultaneously by kikuchi and fujimoto. Kikuchi disease genetic and rare diseases information. Kikuchi fujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare benign, selflimiting cervical lymphadenitis of unknown etiology. To report a case of atypical kikuchi fujimoto disease kfd that illustrates several overlapping features with systemic lupus erythematosus sle. Ferry, in diagnostic pathology of infectious disease second edition, 2018. Causative agents of kikuchi fujimoto disease histiocytic necrotizing lymphadenitis. Also called histiocytic necrotizing lymphadenitis, kikuchifujimoto disease initially described in japan and other asian countries. 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. Kikuchifujimoto disease kfd, necrotizing lymphadenitis is defined pathologically by enlarged lymph nodes with. This disease has now been recognized in many other countries and has also been referred to as kikuchi s disease, histiocytic necrotizing lymphadenitis, focal histio.
Necrosis of lymph node tissue is caused by apoptosis and may be virally induced. Kikuchi fujimoto disease and systemic lupus erythematosus. 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. Reprints and eprints sponsored supplements branded books. Kikuchifujimoto disease orphanet journal of rare diseases. A handful of fatal cases have been described in addition to sparse reports of empirical treatment with a diverse selection of agents, which include antimicrobial and anti. Fujimoto disease kfd is a rare, benign, generally selflimiting disease that has higher prevalence in asian people with a few cases reported in european countries. Two cases are reported in the japanese literature 1 and one from india. Kikuchi fujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is an extremely rare, selflimiting disorder which typically presents with fever and painful, unilateral cervical lymphadenopathy in previously healthy individuals. Although the disease has been reported from all over the world and more so from asia, it is rare.
Also called histiocytic necrotizing lymphadenitis, kikuchi fujimoto disease initially described in japan and other asian countries. A type of rare necrotizing lymphadenitis and its differential diagnosis 187 where a viral origin is assumed, are kawasaki disease and kikuchi fujimoto disease histiocytic necrotizing lymphadenitis. It predominantly affects young women and can closely mimic infective and immunological disorders. Kikuchifujimoto disease with prolonged fever in children. Kikuchifujimoto disease with lymph node, spleen, and liver. It was described in 1972 in japan by kikuchi and fujimoto in dependently 7,8. 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. Kikuchi fujimoto disease is so named because kikuchi and fujimoto were the first scientists to describe it in japan in 1972. A lymph node biopsy showed the features of kikuchi s disease, with necrosis and histiocytic infiltration without neutrophils.
Symptomatic treatment was provided and an uneventful full recovery was made. 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. Kikuchifujimoto disease is so named because kikuchi and fujimoto were the first scientists to describe it in japan in 1972. Histological analysis later suggested kikuchi fujimoto disease, also known as histiocytic necrotising lymphadenitis. An uncommon presentation of kikuchi fujimoto disease. Masahiro kikuchi was born kitakyushu, fukuoka, japan, to a humble family. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting benign lymphadenopathy of unknown origin that predominantly affects young adults. A case of a 55 year old gentleman is described here. Pdf kikuchifujimoto disease in patients with sjogrens syndrome. Kikuchifujimoto disease in a 30yearold caucasian female. Clipping is a handy way to collect important slides you want to go back to later.
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