First described in 1972, kd is increasingly recognized in the literature. Sep 14, 2016 kikuchi disease is not thought to be inherited. This is a benign and selflimited disease, frequently associated with mild fever, and occasionally with other systemic symptoms. Histologically, the dermatological manifestations of kikuchi fujimoto disease have features in common with systemic lupus erythematosus. 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. Necrosis of lymph node tissue is caused by apoptosis and may be virally induced. Histological analysis later suggested kikuchifujimoto disease, also known as histiocytic necrotising lymphadenitis. To report a case of atypical kikuchifujimoto disease kfd that illustrates several overlapping features with systemic lupus erythematosus sle.
It commonly presents with cervical lymphadenitis and fever. 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. Hnl usually manifests as isolated cervical lymphadenopathy accompanied by fever and night sweats. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchi fujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation. Kikuchifujimoto disease with lymph node, spleen, and. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchifujimoto disease, is an uncommon cause of lymphadenopathy. 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 fujimoto disease kfd, a rare clinicopathological entity, is a benign and selflimiting disease. The following cases have burn reported for clinical interest and for the rarity of. 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%. 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. The following cases have burn reported for clinical interest and for the rarity of the reports about this condition in ent literature. 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. Lactate dehydrogenase and alanine aminotransferase concentrations can be raised. Fineneedle aspiration cytology of kikuchi fujimoto disease. 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. Pathology vol 50, issue 6, pages 591698 october 2018.
Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Ct and mr findings dong gyu na, taesub chung, hong sik byun, hong dae kim, young hyeh ko, and jeong hwan yoon summary. Textbook of autoantibodies, 2nd edition, elsevier, am. Mar 22, 2014 the disease is characterised by a unilateral posterior cervical lymphadenopathy. We want to help promote all of your pathology related books. Kikuchifujimoto disease or histiocytic necrotizing. Kikuchi fujimoto disease is a rare, selflimited, histiocytic, necrotizing lymphadenitis first described in japan in 1972. We report a 25 yr old female who presented with fever, polyarthritis and cervical lymphadenopathy. Atlas of lymph node pathology roberto miranda springer. Article pdf available in open journal of pathology 0301. Histologically, the dermatological manifestations of kikuchifujimoto disease have features in common with systemic lupus erythematosus. Kikuchifujimoto disease kfd, also known as kikuchi disease, is a rare lymphohistiocytic disorder first described in 1972.
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. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchifujimoto disease. Webpathology is a free educational resource with 10197 high quality pathology images of benign and malignant neoplasms and related entities. Kikuchifujimoto disease orphanet journal of rare diseases. The trip database provides clinical publications about evidence. Kikuchi disease symptoms and treatment verywell health. Symptomatic treatment was provided and an uneventful full recovery was made.
It predominantly affects young women and can closely mimic infective and immunological disorders. The medicine forum volume 17 article 17 2016 kikuchi disease. Histiocytic necrotizing lymphadenitis kikuchis disease a. To my knowledge, it is the first case of kikuchis disease to be reported in bahrain. It most commonly affects asian adult females younger than 40 years of age. Pathogenesis, diagnosis, and management of kikuchi. 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. 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 kfd, or histiocytic necrotizing lymphadenitis, is a rare, benign, and selflimited disease of unknown etiology that causes lymphadenopathy and has a characteristic. Enigmatic kikuchifujimoto disease american journal of.
Kikuchifujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare benign, selflimiting cervical lymphadenitis of unknown etiology. Ferry, in diagnostic pathology of infectious disease second edition, 2018. Two cases of kikuchi disease showed variable nodal enhancing features, including homogeneous enhancement and focal or extensive nodal necrosis on contrastenhanced ct scans. Necrotizing lymphadenitis, kikuchifujimoto disease. Department of pathology, college of medicine, hanyang university, 17 haengdangdong, seongdonggu, seoul 3792, korea tel. Kikuchi disease, is a rare pathology of the lymph nodes. The disease is characterised by a unilateral posterior cervical lymphadenopathy. It was first described in 1972 by kikuchi and fujimoto in japan independently. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, kikuchi. Here are links to possibly useful sources of information about kikuchi disease. Kfd has a worldwide distribution, and asiatic people have a higher prevalence. Pathogenesis, diagnosis, and management of kikuchi fujimoto disease darcie deaver, phd, pedro horna, md, hernani cualing, md, and lubomir sokol, md, phd. Kikuchi disease genetic and rare diseases information. Ferry, in diagnostic pathology of infectious disease second edition.
To report a case of atypical kikuchi fujimoto disease kfd that illustrates several overlapping features with systemic lupus erythematosus sle. The cause of this disease is not known, although infectious and autoimmune causes have been. Clipping is a handy way to collect important slides you want to go back to later. Kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among. Kikuchifujimoto disease is an extremely rare, benign, and selflimiting. Kikuchi fujimoto disease kfd was described in 1972 as lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris. 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. 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. Institute of pathology, queen elizabeth hospital, hong kong. Kikuchifujimoto disease is a rare, selflimited, histiocytic, necrotizing lymphadenitis first described in japan in 1972. Typically it runs a benign course and appears to resolve spontaneously one to six months after definitive diagnosis. Now customize the name of a clipboard to store your clips.
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. Kikuchifujimoto disease is a rare benign cervical lymphadenopathy, which often affects young adult women. Kikuchifujimoto disease is a rare benign, condition of necrotising histiocytic lymphadenitis. This disease is of unknown etiology, but an infectious etiology has been implicated in the. Most cases occur sporadically in people with no family history of the condition. While most common in southeast asia, where kfd was initially described in 1972 in young women, this lesion has since been. Kikuchis disease of the neck histiocytic necrotizing lymphadenitis. 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. Kikuchifujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence. An atypical presentation of kikuchifujimoto disease. Robbins basic pathology 10th edition pdf free download. We retrieved 6 cases of hnl with available archival frozen tissues from the pathology files of the brigham and womens hospital, boston, ma.
Kikuchifujimoto disease kfd, a rare clinicopathological entity, is a benign and selflimiting disease. Kikuchifujimoto disease is an extremely rare, benign, and self limiting. It is mainly a disease of young adults 2030 years, with a slight bias towards females. Kikuchifujimoto disease kfd also known as histiocytic necrotizing lymphadenitis. 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. Kikuchis disease of the mesenteric lymph nodes presenting as. It most commonly affects adults younger than 40 years of age and of asian descent. Kikuchifujimoto disease in a 30yearold caucasian female.
Kikuchi fujimoto disease is a rare benign, condition of necrotising histiocytic lymphadenitis. Sep 25, 2018 in patients with kikuchi disease, diagnostic laboratory and radiologic test findings are nonspecific. A 16yearold boy with an unusual manifestation of kikuchifujimoto disease kfd is described. Kikuchifujimoto disease, also known as subacute necrotizing lymphadenitis or subacute necrotizing histiocytosis, is an idiopathic disease characterized usually by cervical lymph node enlargement 80%. If you have problems viewing pdf files, download the latest version of adobe reader. Kikuchi fujimoto disease was diagnosed after cervical lymph node biopsy. 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. Masahiro kikuchi was born kitakyushu, fukuoka, japan, to a humble family. Kikuchi fujimoto disease is a benign and selflimited disease with a female predominance affecting preferentially the asian population. Kikuchifujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is a relatively rare condition characterized by subacute necrotizing regional lymphadenopathy. 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 radiology reference article.
Kikuchifujimoto disease with lymph node, spleen, and liver. Histiocytic necrotizing lymphadenitis hnl is a rare benign disorder. 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. Kikuchis disease, lymphadenitis, lymphoma, systemic lupus erythematosus. Kikuchi disease is fairly common in young people, predominantly young women, in asia. Jun 10, 2005 kikuchi fujimoto disease is a rare benign cervical lymphadenopathy, which often affects young adult women. Despite its low incidence, kikuchifujimoto disease should be considered in patients with persistent lymphadenopathy. He had it in his neck in february 2017 it was removed and he got better.
Kikuchi disease kd is an enigmatous, selflimiting, rarely fatal, disease of young females. Kikuchis disease nord national organization for rare. Kikuchi fujimoto disease is a rare lymphohistiocytic disorder that affects young women of asian descent more frequently than persons of other ethnic groups. It effects cervical lymph nodes, predominantly in young females. A rare lymph node disorder which is not cancerous but causes large, inflamed lymph nodes. 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. Kikuchis disease may be seen in association with lupus, stills disease, interstitial lung disease, hemophagocytic syndrome, mononeuritis, or infections e. Despite its low incidence, kikuchi fujimoto disease should be considered in patients with persistent lymphadenopathy.
The selflimiting disorder usually occurs in asian women in their late 20s or early 30s. From the section of hematopathology, department of pathology. 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. Now he has a softball size lymph node in his abdomen that the drs think might be. Involved lymph nodes demonstrate paracortical areas. Reprints and eprints sponsored supplements branded books about. Recognizing a common presentation of an uncommon condition ramya punati, md thomas jefferson university, ramya. Less frequent symptoms include weight loss, nausea, vomiting, sore throat. Kikuchi fujimoto disease also known as histiocytic necrotizing lymphadenitis is an idiopathic benign self limiting condition with a favourable outcome, usually affecting young women. 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. Kikuchi disease symptoms, diagnosis, treatments and causes.
Kikuchi disease is characterized histopathologically by. Now he has a softball size lymph node in his abdomen that the drs think might be kikuchi disease again. The disease was described in 1972 by kikuchi 1 and independently by fujimoto et al 2. More detailed information about the symptoms, causes, and treatments of kikuchi disease is available below symptoms of kikuchi disease. 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. Histiocytic necrotizing lymphadenitis kikuchifujimoto disease. 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. Pdf kikuchifujimoto disease in patients with sjogrens syndrome. Kikuchi disease kd, also known as kikuchifujimoto disease or histiocytic necrotizing lymphadenitis, is a selflimiting cause of cervical lymphadenopathy. 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. The exact cause of the disease is not yet known, although some researchers have suggested it is an infection or autoimmune disorder.
Kikuchis disease is characterized by lymphadenopathy in young patients and may be mistaken for malignant disease both clinically and. Kikuchi first described the disease in 1972 in japan. He presented with fevers, weight loss and tender cervical lymph nodes. It is also known as histiocytic necrotising lymphadenitis, abbreviated hnl, and kikuchifujimoto disease.
For language access assistance, contact the ncats public information officer. It is well described in the pathology literature, but little is written about this in the ent literature, as. Kikuchis disease of the mesenteric lymph nodes presenting. Histological analysis later suggested kikuchi fujimoto disease, also known as histiocytic necrotising lymphadenitis. Kikuchis disease is a very rare disease mainly seen in japan.
Although mesenteric lymph node involvement of kikuchis disease is very rare, kikuchis disease should be added to the. Kikuchifujimoto disease kfd is a rare entity charac terized by subacute. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation. Histiocytic necrotizing lymphadenitis kikuchifujimoto.
Our previous reports showed detection of antipituitary antibodies apa by immunoblot analysis and. Kikuchifujimoto disease, or histiocytic necrotizing lymphadenitis, is a selflimited condition, characterized by benign lymphadenopathy with associated fevers and systemic symptoms. 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. Kikuchifujimoto disease was diagnosed after cervical lymph node biopsy. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting benign lymphadenopathy of unknown origin that predominantly affects young adults. There is a higher incidence of kfd in women aged 2035 years and in asian populations. The exact etiology and the pathogenesis of kikuchis disease is unknown, but the clinical. Isolated cases are reported in north america, europe, asia and at least two cases new zealand. Pathogenesis, diagnosis, and management of kikuchifujimoto. 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.
Ideal sources for wikipedia s health content are defined in the guideline wikipedia. A case of a 55 year old gentleman is described here. Kikuchis disease is a rare nonmalignant disorder that affects the lymph nodes. Sep 25, 2018 kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchi fujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Immunohistochemistry may be necessary to distinguish kikuchis from malignant lymphoma. Kikuchifujimoto disease kfd was described in 1972 as lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris.
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