We report a 25 yr old female who presented with fever, polyarthritis and cervical lymphadenopathy. Kikuchis disease is a very rare disease mainly seen in japan. It commonly presents with cervical lymphadenitis and fever. Recognizing a common presentation of an uncommon condition ramya punati, md thomas jefferson university, ramya. Now customize the name of a clipboard to store your clips. There is a higher incidence of kfd in women aged 2035 years and in asian populations. More detailed information about the symptoms, causes, and treatments of kikuchi disease is available below symptoms of kikuchi disease. Kikuchis disease, lymphadenitis, lymphoma, systemic lupus erythematosus. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Kikuchifujimoto disease is an extremely rare, benign, and selflimiting. The selflimiting disorder usually occurs in asian women in their late 20s or early 30s.
While most common in southeast asia, where kfd was initially described in 1972 in young women, this lesion has since been. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting benign lymphadenopathy of unknown origin that predominantly affects young adults. Kikuchifujimoto disease kfd, also known as histiocytic lymphadenitis, is a benign, selflimiting disease that manifests primarily as cervical lymphadenopathy but may include lowgrade fever, headache, and fatigue. Kikuchi disease kd is an enigmatous, selflimiting, rarely fatal, disease of young females. Kikuchis disease of the mesenteric lymph nodes presenting as. Kikuchifujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence. Reprints and eprints sponsored supplements branded books about. Lymph nodes are present throughout the body as small oval structures that filter lymph fluid, fight infection and form white blood cells and blood plasma cells.
It was first described in 1972 by kikuchi and fujimoto in japan independently. 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 disease is characterised by a unilateral posterior cervical 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 kfd, also known as kikuchi disease, is a rare lymphohistiocytic disorder first described in 1972. Kikuchi fujimoto disease is a benign and selflimited disease with a female predominance affecting preferentially the asian population. It effects cervical lymph nodes, predominantly in young females. Takashi kikuchi, shigeki yabe, tsugiyasu kanda and isao kobayashi department of laboratory medicine and clinical laboratory center, gunma university school of medicine, 33915, showamachi, maebashi 3718511, japan abstract. 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. The trip database provides clinical publications about evidence. Kikuchis disease is a fairly common selflimited disorder among orientals that usually involves the cervical lymph nodes of young individuals and occurs predominantly in females. The following cases have burn reported for clinical interest and for the rarity of the reports about this condition in ent literature.
Kikuchifujimoto disease kfd also known as histiocytic necrotizing lymphadenitis. Sep 25, 2018 kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchi fujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. 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. Kikuchi disease is characterized histopathologically by. Pathogenesis, diagnosis, and management of kikuchi. Kikuchifujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare, benign, and selflimited disease of unknown etiology that causes lymphadenopathy and has a characteristic. Kikuchifujimoto disease radiology reference article. Histiocytic necrotizing lymphadenitis kikuchifujimoto disease. It predominantly affects young women and can closely mimic infective and immunological disorders.
Recently, a case study reporting two familial cases of kikuchi disease was published in the literature, raising the hypothesis of genetic factors or perhaps a predisposing genetic background. A rare lymph node disorder which is not cancerous but causes large, inflamed lymph nodes. Now he has a softball size lymph node in his abdomen that the drs think might be kikuchi disease again. This is a benign and selflimited disease, frequently associated with mild fever, and occasionally with other systemic symptoms. To my knowledge, it is the first case of kikuchis disease to be reported in bahrain. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, kikuchi. 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. The medicine forum volume 17 article 17 2016 kikuchi disease. Kikuchifujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare benign, selflimiting cervical lymphadenitis of unknown etiology.
Sep 14, 2016 kikuchi disease is not thought to be inherited. Kikuchi disease is fairly common in young people, predominantly young women, in asia. Article pdf available in open journal of pathology 0301. Hnl usually manifests as isolated cervical lymphadenopathy accompanied by fever and night sweats. Kikuchi disease symptoms and treatment verywell health. Kikuchifujimoto disease was diagnosed after cervical lymph node biopsy.
Ct and mr findings dong gyu na, taesub chung, hong sik byun, hong dae kim, young hyeh ko, and jeong hwan yoon summary. Kikuchi s disease is a fairly common selflimited disorder among orientals that usually involves the cervical lymph nodes of young individuals and occurs predominantly in females. 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. 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 22, 2014 the disease is characterised by a unilateral posterior cervical lymphadenopathy. Institute of pathology, queen elizabeth hospital, hong kong. Jun 10, 2005 kikuchi fujimoto disease is a rare benign cervical lymphadenopathy, which often affects young adult women. Masahiro kikuchi was born kitakyushu, fukuoka, japan, to a humble family. Kikuchis disease of the neck histiocytic necrotizing lymphadenitis. Dec 09, 2017 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. Kikuchi fujimoto disease kfd, necrotizing lymphadenitis is defined pathologically by enlarged lymph nodes with characteristic microscopic findings and nearly always follows a selflimiting benign clinical course. Involved lymph nodes demonstrate paracortical areas. Histiocytic necrotizing lymphadenitis kikuchifujimoto.
Kikuchifujimoto disease in a 30yearold caucasian female. Ferry, in diagnostic pathology of infectious disease second edition. Enigmatic kikuchifujimoto disease american journal of. Textbook of autoantibodies, 2nd edition, elsevier, am. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchifujimoto disease, is an uncommon cause of lymphadenopathy. Atlas of lymph node pathology roberto miranda springer. Pathogenesis, diagnosis, and management of kikuchi fujimoto disease darcie deaver, phd, pedro horna, md, hernani cualing, md, and lubomir sokol, md, phd.
Despite its low incidence, kikuchi fujimoto disease should be considered in patients with persistent lymphadenopathy. Most cases occur sporadically in people with no family history of the condition. Here are links to possibly useful sources of information about kikuchi disease. Histiocytic necrotizing lymphadenitis kikuchis disease a. Necrosis of lymph node tissue is caused by apoptosis and may be virally induced. Kikuchi disease genetic and rare diseases information.
The cause of this disease is not known, although infectious and autoimmune causes have been. It most commonly affects asian adult females younger than 40 years of age. Kikuchi first described the disease in 1972 in japan. For language access assistance, contact the ncats public information officer. Histologically, the dermatological manifestations of kikuchi fujimoto disease have features in common with systemic lupus erythematosus. Fineneedle aspiration cytology of kikuchi fujimoto disease. Kikuchi disease, is a rare pathology of the lymph nodes.
Kikuchifujimoto disease is a rare benign cervical lymphadenopathy, which often affects young adult women. We want to help promote all of your pathology related books. 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 under michio hashimoto. Two cases of kikuchi disease showed variable nodal enhancing features, including homogeneous enhancement and focal or extensive nodal necrosis on contrastenhanced ct scans. Kikuchifujimoto disease orphanet journal of rare diseases.
Less frequent symptoms include weight loss, nausea, vomiting, sore throat. Kikuchifujimoto disease with lymph node, spleen, and. Kikuchifujimoto disease is a rare, selflimited, histiocytic, necrotizing lymphadenitis first described in japan in 1972. Although mesenteric lymph node involvement of kikuchis disease is very rare, kikuchis disease should be added to the. Kikuchifujimoto disease or histiocytic necrotizing. Histological analysis later suggested kikuchi fujimoto disease, also known as histiocytic necrotising lymphadenitis. Necrotizing lymphadenitis, kikuchifujimoto disease. Pdf kikuchifujimoto disease in patients with sjogrens syndrome. Kikuchifujimoto disease kfd, a rare clinicopathological entity, is a benign and selflimiting disease. The exact cause of the disease is not yet known, although some researchers have suggested it is an infection or autoimmune disorder.
Sep 25, 2018 in patients with kikuchi disease, diagnostic laboratory and radiologic test findings are nonspecific. Pathogenesis, diagnosis, and management of kikuchifujimoto. 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. The exact etiology and the pathogenesis of kikuchis disease is unknown, but the clinical. Kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among. It is also known as histiocytic necrotising lymphadenitis, abbreviated hnl, and kikuchifujimoto disease. Kikuchis disease of the mesenteric lymph nodes presenting as acute appendicitis corresponding author seung sam paik, m. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Kikuchi fujimoto disease is a rare benign, condition of necrotising histiocytic lymphadenitis. Typically it runs a benign course and appears to resolve spontaneously one to six months after definitive diagnosis. Ideal sources for wikipedia s health content are defined in the guideline wikipedia. Histologically, the dermatological manifestations of kikuchifujimoto disease have features in common with systemic lupus erythematosus. Kikuchifujimoto disease, also known as subacute necrotizing lymphadenitis or subacute necrotizing histiocytosis, is an idiopathic disease characterized usually by cervical lymph node enlargement 80%. Clipping is a handy way to collect important slides you want to go back to later.
Histological analysis later suggested kikuchifujimoto disease, also known as histiocytic necrotising lymphadenitis. A case of a 55 year old gentleman is described here. An atypical presentation of kikuchifujimoto disease. It is mainly a disease of young adults 2030 years, with a slight bias towards females. To report a case of atypical kikuchifujimoto disease kfd that illustrates several overlapping features with systemic lupus erythematosus sle. Now he has a softball size lymph node in his abdomen that the drs think might be. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchi fujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation. Kikuchis disease nord national organization for rare.
Kikuchifujimoto disease kfd is a benign and selflimited disorder, characterized by regional cervical lymphadenopathy with tenderness, usually accompanied with mild fever and night sweats. Department of pathology, college of medicine, hanyang university, 17 haengdangdong, seongdonggu, seoul 3792, korea tel. Despite its low incidence, kikuchifujimoto disease should be considered in patients with persistent lymphadenopathy. 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. We present the case of kikuchifujimoto disease in the polish population and analyse the difficulties in differentiating this disease from the systemic lupus erythematosus. Kikuchifujimoto disease, or histiocytic necrotizing lymphadenitis, is a selflimited condition, characterized by benign lymphadenopathy with associated fevers and systemic symptoms. This disease is of unknown etiology, but an infectious etiology has been implicated in the. First described in 1972, kd is increasingly recognized in the literature. Kikuchifujimoto disease with lymph node, spleen, and liver. Pathology vol 50, issue 6, pages 591698 october 2018. Kikuchi fujimoto disease is a rare lymphohistiocytic disorder that affects young women of asian descent more frequently than persons of other ethnic groups.
Kikuchi fujimoto disease is a rare, selflimited, histiocytic, necrotizing lymphadenitis first described in japan in 1972. Kikuchi disease kd, also known as kikuchifujimoto disease or histiocytic necrotizing lymphadenitis, is a selflimiting cause of cervical lymphadenopathy. Kikuchifujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is a relatively rare condition characterized by subacute necrotizing regional lymphadenopathy. 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. The following cases have burn reported for clinical interest and for the rarity of. We retrieved 6 cases of hnl with available archival frozen tissues from the pathology files of the brigham and womens hospital, boston, ma.
Symptomatic treatment was provided and an uneventful full recovery was made. If you have problems viewing pdf files, download the latest version of adobe reader. Kikuchifujimoto disease kfd was described in 1972 as lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris. 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. Ferry, in diagnostic pathology of infectious disease second edition, 2018. Kikuchi fujimoto disease was diagnosed after cervical lymph node biopsy.
Kikuchi fujimoto disease kfd, a rare clinicopathological entity, is a benign and selflimiting disease. Kfd has a worldwide distribution, and asiatic people have a higher prevalence. Our previous reports showed detection of antipituitary antibodies apa by immunoblot analysis and. May 23, 2006 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. He had it in his neck in february 2017 it was removed and he got better. 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. Lactate dehydrogenase and alanine aminotransferase concentrations can be raised. It is well described in the pathology literature, but little is written about this in the ent literature, as. Webpathology is a free educational resource with 10197 high quality pathology images of benign and malignant neoplasms and related entities.
Kikuchifujimoto disease kfd is a rare entity charac terized by subacute. Robbins basic pathology 10th edition pdf free download. Kikuchis disease is characterized by lymphadenopathy in young patients and may be mistaken for malignant disease both clinically and. He presented with fevers, weight loss and tender cervical lymph nodes. Kikuchifujimoto disease is an extremely rare, benign, and self limiting.
To report a case of atypical kikuchi fujimoto disease kfd that illustrates several overlapping features with systemic lupus erythematosus sle. Although results of fineneedle aspiration fna of an affected lymph node may be suggestive, 30, 31 the diagnosis of kikuchi disease is confirmed only by excisional lymph node biopsy. Kikuchifujimoto disease is a rare benign, condition of necrotising histiocytic lymphadenitis. Kikuchis disease of the mesenteric lymph nodes presenting. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchifujimoto disease. Kikuchi fujimoto disease also known as histiocytic necrotizing lymphadenitis is an idiopathic benign self limiting condition with a favourable outcome, usually affecting young women. Histiocytic necrotizing lymphadenitis hnl is a rare benign disorder. Isolated cases are reported in north america, europe, asia and at least two cases new zealand. The disease was described in 1972 by kikuchi 1 and independently by fujimoto et al 2. Kikuchi disease symptoms, diagnosis, treatments and causes. Below are the technical specifications of robbins basic pathology 10th edition pdf. Immunohistochemistry may be necessary to distinguish kikuchis from malignant lymphoma. From the section of hematopathology, department of pathology. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation.
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