The cardinal symptoms are fever, lymphadenopathy and night sweat. Cytologic examination by fine needle aspiration fna can suggest the diagnosis of kikuchi disease, especially when supported by typical clinical findings, but excisional biopsy of an involved lymph node is needed to confirm the diagnosis in doubtful cases. Original article usefulness of fluorodeoxyglucosepositron. The differential diagnosis of tuberculosis meningitis is opportune and has been thoroughly discussed. Kikuchifujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a benign and selflimited disease that mainly affects young women. Kikuchi disease, is a rare pathology of the lymph nodes. The trip database provides clinical publications about evidence. Histiocytic necrotizing lymphadenitis, or kikuchis disease, is a selflimiting cervical lymphadenitis of unknown origin. Antinuclear antibodies, antiphospholipid antibodies, antidsdna, and rheumatoid factor are usually negative, and may help in differentiation from systemic lupus erythematosus. Dec 09, 2017 while im sure you know a lot about kikuchi disease already, i did a little research and found some information on a rare diseases website. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchi fujimoto disease. Kikuchi disease kd or histiocytic necrotizing lymphadentitis is a rare cause of lymphadenopathy. Sep 25, 2018 a definitive diagnosis of kikuchi disease can be made only by tissue evaluation. Kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with a higher prevalence among japanese and other asiatic individuals.
The simultaneous occurrence of kikuchis lymphadenitis with carcinoma is unusual and presents an interesting. More detailed information about the symptoms, causes, and treatments of kikuchi disease is available below. The chest radiograph revealed double lower lobe infiltrate, which was unresponsive to antibiotics. Uptodate, electronic clinical resource tool for physicians and patients that provides information on adult primary care and internal medicine, allergy and immunology, cardiovascular medicine, emergency medicine, endocrinology and diabetes, family medicine, gastroenterology and hepatology, hematology, infectious diseases, nephrology and hypertension, neurology, obstetrics, gynecology, and women. Kikuchi disease genetic and rare diseases information center. Kikuchi fujimoto disease is a rare, selflimited, histiocytic, necrotizing lymphadenitis first described in japan in 1972. Fujimoto and colleagues independently described kikuchis disease in the same year. Aseptic meningitis in kikuchi fujimoto disease is described in the literature but is rare. 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. We characterized the clinical and ct findings in a large group of patients with kd. Kikuchifujimoto disease kfd is a benign and selflimited disorder, characterized by regional cervical lymphadenopathy with tenderness, usually accompanied with mild fever and night sweats. Kikuchi disease is a benign noncancerous condition of the lymph nodes.
Case report kikuchis lymphadenitis in inguinal lymph nodes with. Kfd can occur in association with systemic lupus erythematosus. Kikuchis disease nord national organization for rare. Pdf pathogenesis, diagnosis, and management of kikuchi. Kikuchi disease is an uncommon and generally selflimited cause of infected lymph nodes lymphadenitis that usually resolves in several weeks to months.
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. Kikuchi fujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a benign and selflimited disease that mainly affects young women. It was first described in 1972 by kikuchi and fujimoto in japan independently. 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. Less frequent symptoms include weight loss, nausea, vomiting, sore throat. Kikuchi first described the disease in 1972 in japan. Kikuchifujimoto disease is a rare, selflimited, histiocytic, necrotizing lymphadenitis first described in japan in 1972. Severe kikuchis disease responsive to immune modulation. Kikuchifujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare benign, selflimiting cervical lymphadenitis of unknown etiology. Despite its low incidence, kikuchifujimoto disease should be considered in patients with persistent lymphadenopathy.
Kikuchifujimoto disease kfd is a form of histiocytic necrotizing. Kikuchifujimoto disease in a 30yearold caucasian female. Kikuchi disease symptoms, diagnosis, treatments and causes. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting benign lymphadenopathy of unknown origin that predominantly affects young adults. Ideal sources for wikipedia s health content are defined in the guideline wikipedia. You probably already know most of the information, but thought it might be of interest to you. Kikuchi fujimoto disease presenting as pyrexia of unknown origin alladi mohan, 1 j.
Sep 25, 2018 kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchi fujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. This may precede, coincide with or develop after the presentation of kikuchis disease 5, 6. Management of kikuchifujimoto disease using glucocorticoid. It commonly presents with cervical lymphadenitis and fever. The initial description was referred as kikuchis and fujimoto disease or. Kikuchifujimoto disease kfd is a histiocytic necrotising lymphadenitis, which is a benign disease of unknown aetiology. Although mesenteric lymph node involvement of kikuchis disease is very rare, kikuchis disease should be added to the. Kikuchifujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting, benign, and rare systemic lymphadenitis with unknown etiology. Causative agents of kikuchi fujimoto disease histiocytic necrotizing lymphadenitis.
Kikuchifujimoto disease presenting as puo mohan et al. My 30 year old son has been but there is so little information on it and the drs he has seen its the first time they have heard of it. A collection of disease information resources and questions answered by our. If you have problems viewing pdf files, download the latest version of adobe reader. To our knowledge, there is no previous study comparing the clinical and laboratory characteristics of patients from different geographical parts of the world. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Kikuchifujimoto disease radiology reference article.
Patients present with localized lymphadenopathy, fever, and leukopenia in up to half of the cases. First described independently by kikuchi 27 and fujimoto 19 in 1972, kikuchifujimoto disease is increasingly recognized in the literature. He had it in his neck in february 2017 it was removed and he got better. Laboratory test abnormalities include elevated erythrocyte sedimentation rate esr, and white blood count abnormalities low neutrophil count and elevated lymphocyte count with atypical lymphocytes in the peripheral blood. Kikuchifujimoto disease, also known as kikuchis disease or histiocytic necrotizing lymphadenitis, is a benign, selflimiting cause of lymphadenopathy, variably associated with systemic symptoms. Despite its low incidence, kikuchi fujimoto disease should be considered in patients with persistent lymphadenopathy. The association of kikuchi fujimoto disease kfd with cryptogenic organizing pneumonia cop is extremely rare. Kikuchifujimoto disease, also known as subacute necrotizing lymphadenitis or subacute necrotizing histiocytosis, is an idiopathic disease characterized usually by cervical lymph node enlargement 80%. This disorder is often mistaken for malignant lymphoma, especially cervical adenopathy because the symptoms are very similar. Kikuchifujimoto disease kfd, a rare clinicopathological entity, is a benign and selflimiting disease. Kikuchi fujimoto disease in patient with systemic phacomatosis pigmentovascularis. Kikuchifujimoto disease orphanet journal of rare diseases. Kikuchi fujimoto disease associated with cryptogenic.
Kikuchis disease and systemic lupus erythematosus in a. If you have problems viewing pdf files, download the latest version of adobe. For language access assistance, contact the ncats public information officer. Kikuchifujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence. Kikuchis disease of the mesenteric lymph nodes presenting. Sep 14, 2016 kikuchi disease is a benign noncancerous condition of the lymph nodes.
The exact cause of the disease is not yet known, although some researchers have suggested it is an infection or autoimmune disorder. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchifujimoto disease, is an uncommon cause of lymphadenopathy. Less common symptoms include weight loss, nausea, vomiting, and sore throat. Kikuchifujimoto disease kfd is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. It predominantly affects young women and can closely mimic infective and immunological disorders. Less frequent symptoms include weight loss, nausea, vomiting, sore. Kikuchifujimoto disease kfd was first described in japan in 1972. We report a case of simultaneous diagnosis of kfd and cop. The aim of this study was to analyze the diagnostic pathway of kd and clarify the usefulness of fluorodeoxyglucosepositron emission tomographycomputed tomography 18ffdg petct. A rare lymph node disorder which is not cancerous but causes large, inflamed lymph nodes. Pathogenesis, diagnosis, and management of kikuchifujimoto. Phaneendra 2 departments of 1 medicine, 2 pathology, sri venkateswara institute of medical sciences, tirupati. Kikuchis disease was recognized for the first time as a separate syndrome or disease in japan by kikuchis and fujimoto independently in two separate cases among young woman in 1972. Kikuchifujimoto disease kfd also known as histiocytic necrotizing lymphadenitis.
Kikuchifujimoto disease kfd is a rare, selflimiting disease of unknown aetiology presenting with cervical lymphadenopathy, fever, vomiting, weight loss, night sweats and chills. The disease was described in 1972 by kikuchi 1 and independently by fujimoto et al 2. The illness in may 1999 was similar to the present one and so this case represents a case of recurrent kikuchi fujimoto. Prasad2 departments of 1paediatrics, 2pathology, sri devraj urs medical college, kolar abstract kikuchi fujimoto disease kfd is a rare, selflimiting disease of unknown aetiology presenting with cervical. While the exact cause of this condition is unknown, infectious and autoimmune causes have been suggested. Dec, 2005 kikuchi s disease is an idiopathic illness characterized by a selflimiting lymphadenitis that normally resolves over subsequent weeks or months without specific treatment. Kikuchifujimoto disease is an extremely rare, benign, and self limiting. Now he has a softball size lymph node in his abdomen that the drs think might be. Kikuchi s disease, also known as histiocytic necrotizing lymphadenopathy, is a rare, benign, noncancerous, nonmalignant disorder of the lymph nodes of young adults, predominantly of young women.
Common symptoms reported by people with kikuchi disease. A disorder that typically causes swollen glands in the neck cervical lymphadenopathy together with fever or flulike symptoms. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchi fujimoto disease, is an uncommon cause of lymphadenopathy. The exact etiology and the pathogenesis of kikuchis disease is unknown, but the clinical. A 12yearold female suffered from cervical lymphadenopathy, leukocytopenia, fever and especially skin rash. The disease frequently mimics tuberculous lymphadenitis, malign lymphoma, and many other benign and malignant conditions. Kikuchi disease genetic and rare diseases information. Dec 31, 2015 kikuchi s disease is an idiopathic illness characterized by a selflimiting lymphadenitis that normally resolves over subsequent weeks or months without specific treatment. Kikuchifujimoto disease is rare in children, however, it is well documented in the literature. Clipping is a handy way to collect important slides you want to go back to later. Kikuchi disease is characterized histopathologically by.
Kikuchis disease and systemic lupus erythematosus in a saudi child annals of saudi medicine, vol 18 no. Kikuchifujimoto disease kfd, also known as kikuchi disease, is a rare lymphohistiocytic disorder first described in 1972. Kikuchi fujimoto disease kfd or histiocytic necrotizing lymphadenitis is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Kikuchi fujimoto disease kfd is a histiocytic necrotising lymphadenitis, which is a benign disease of unknown aetiology. Kikuchi disease, also called kikuchifujimoto 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. Affected patients are most often young adults under the age of 30 years. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchi fujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation.
Kikuchi disease is a selflimiting illness which has symptoms which may overlap with hodgkins lymphoma leading to misdiagnosis in some patients. Management of kikuchis disease using glucocorticoid the. Histiocytic necrotizing lymphadenitis, kikuchi disease, kikuchifujimoto disease, kikuchi lymphadenitis, lymphadenopathy, kfd kfd is a rare lymphohistiocytic disorder due to cervical inflammatory lymphadenitis or axillary region and even other location seldom with an unknown etiopathogenesis, that is. Also called kikuchifujimoto disease or histiocytic necrotizing lymphadenitis, this condition was discovered in japan in 1972 and since then has seen in other areas of the world.
Kikuchifujimoto disease kfd manifests in most cases as unilateral. Browse clinical cases articles from the new england journal of medicine. We report a 25 yr old female who presented with fever, polyarthritis and cervical lymphadenopathy. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. A 12yearold female suffered from cervical lymphadenopathy, leukocytopenia, fever and. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchifujimoto disease. Misdiagnosing kfd as lymphoma or systemic lupus erythematosus is not uncommon due to the similarity of clinical and histopathological features of these diseases. May 23, 2006 kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with a higher prevalence among japanese and other asiatic individuals.
Kikuchi disease symptoms and treatment verywell health. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation. Kikuchi fujimoto disease kfd, a rare clinicopathological entity, is a benign and selflimiting disease. To my knowledge, it is the first case of kikuchis disease to be reported in bahrain. Pdf kikuchifujimoto disease kfd is a benign and selflimited disorder, characterized by regional cervical lymphadenopathy with tenderness, usually. Kikuchis lymphadenitis, carcinoma, coexistent disease. Pathogenesis, diagnosis, and management of kikuchi. 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. 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%. Mar 15, 2006 kikuchifujimoto disease kfd was first described in japan in 1972. 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. Review article kikuchifujimoto disease associated with.
Since no specific treatment has been reported for kikuchis disease to date, once a diagnosis of kikuchis disease has been established, the role of the physician has been limited only to treating the symptoms. Now customize the name of a clipboard to store your clips. Kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among. Between 1990 and 2002, 96 patients 68 women, 28 men. Kikuchi fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting, benign, and rare systemic lymphadenitis with unknown etiology. Kikuchi disease is fairly common in young people, predominantly young women, in asia. Successful treatment of severe kikuchis disease with.
S ir in the 15 october issue of clinical infectious diseases, noursadeghi et al. Review article kikuchifujimoto disease associated with sjogrens syndrome. This disease is of unknown etiology, but an infectious etiology has been implicated in the pathogenesis of at least a subset of cases of kikuchi. The main symptoms include swollen lymph nodes in the neck, mild fever, and night sweats. Two cases are reported in the japanese literature 1 and one from india. A 33yearold male presented with a 1month cough illness and fever lasting for 5 days.