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. 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. Jun 27, 2014 castleman disease is a rare lymphoproliferative disorder of unknown etiology that most commonly presents as a mediastinal nodal mass or, in the extranodal form of the disease, a mass located in the mediastinum or retroperitoneum. Sep 28, 2012 kikuchi disease kikuchifujimoto disease, histiocytic necrotizing lymphadenitis is a systemic illness of unkown etiology. Kikuchifujimoto disease, also known as subacute necrotizing lymphadenitis or subacute necrotizing histiocytosis, is an idiopathic disease characterized usually by cervical lymph node enlargement 80%. Kikuchi fujimoto disease, an extremely rare cause of lymphadenopathy is histiocytic ecrotizing lymphadenitis. This disease has now been recognized in many other countries and has also been referred to as kikuchi s disease, histiocytic necrotizing lymphadenitis, focal histiocytic.
Kikuchifujimoto disease, known as kikuchi disease, is a rare benign. Melk mediates the stability of ezh2 through sitespecific phosphorylation in extranodal natural killertcell lymphoma. Kikuchi fujimoto disease kfd is a selflimiting disease that has an unknown cause and was first described independently in 1972 by kikuchi 1 and fujimoto et al 2 in japan. First described independently by kikuchi 27 and fujimoto 19 in 1972, kikuchifujimoto disease is increasingly recognized in the literature. The main sign of kikuchi disease is usually swollen lymph nodes in the neck, which tend to develop suddenly. It is mainly a disease of young adults 2030 years, with a slight bias towards females. Kikuchifujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence. Kikuchi disease is fairly common in young people, predominantly young women, in asia. Kikuchifujimoto disease kfd is a selflimited disease characterized by necrotizing lymphadenitis. Diagnosis kikuchifujimoto disease is generally diagnosed on the basis of an excisional biopsy of affected lymph nodes. Kikuchifujmoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is a rare lymphadenitis of unknown origin, but with an excellent prognosis.
Kikuchi disease with skin lesions mimicking lupus erythematosus. To our knowledge, there is no previous study comparing the clinical and laboratory characteristics of patients from different geographical parts of the world. It is a rare cause of lymphadenopathy, commonly seen in individuals of asian descent and frequently associated with fever. It can run a benign course although severe extranodal forms are known to occur, which might warrant systemic immunosuppression. Two cases of kikuchi disease showed variable nodal enhancing features, including homogeneous enhancement and focal or extensive nodal necrosis on contrastenhanced ct scans. Sep 14, 2016 kikuchi disease is not thought to be inherited.
Kikuchifujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is a relatively rare condition characterized by subacute necrotizing regional lymphadenopathy. Histopathology of the involved lymph nodes differentiates kikuchi disease from several more. Kikuchis disease seems to be more prevalent in japanese and asian individuals. Kikuchis disease is a very rare disease mainly seen in japan. Kikuchifujimoto disease kfd is a rare entity charac terized by subacute. Kikuchi disease genetic and rare diseases information. The head and neck region is a common site for extranodal lymphomas, second only to the gastrointestinal tract. Kikuchifujimoto disease kfd or histiocytic necrotising lymphadenitis is a rare, selflimiting, benign syndrome commonly presenting with cervical lymphadenopathy and fever. Kikuchifujimoto disease, also known as kikuchis disease or histiocytic necrotizing lymphadenitis, is a benign, selflimiting cause of lymphadenopathy, variably associated with systemic symptoms. Nonsteroidal antiinflammatory drugs nsaids may be used to alleviate lymph node tenderness and fever.
The skin is the most frequently affected extranodal organ. Kikuchifujimoto disease with prolonged fever in children. Young adults of asian ancestry are most commonly affected, but it has been reported worldwide. Kikuchi disease, is a rare pathology of the lymph nodes. Treatment of kikuchi disease is generally supportive. The cardinal symptoms are fever, lymphadenopathy and night sweat. Kikuchifujimoto disease in a patient with mediastinal. It was first described by kikuchi and fujimoto et al. Kikuchifujimoto disease orphanet journal of rare diseases. Extranodal involvement has also been described, and skin manifestations, such papulopustular lesions, may be observed 2,4. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchi fujimoto disease, is an uncommon cause of lymphadenopathy.
Kikuchis disease nord national organization for rare. Nonhodgkin lymphomas are most common, and hodgkin lymphoma occurs rarely at extranodal sites in the head and neck. 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, 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. It can run a benign course although severeextranodal forms are known to occur, which might warrant systemic. Kikuchi fujimoto disease or histiocytic necrotizing lymphadenitis, is a rare, idiopathic, usually selflimited disease characterized by cervical lymphadenopathy and general symptoms was described for the first time in 1972 by japanese pathologists kikuchi and fujimoto independently. Generalized lymphadenopathy and extranodal involvement are rare. Primarily a disease of young women, it is characterised by cervical lymphadenopathy that resolves spontaneously 1. Kikuchifujimoto disease, an extremely rare cause of lymphadenopathy is histiocytic ecrotizing lymphadenitis. Kikuchifujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a selflimited disease of complex and unclear etiology. Sep 25, 2018 treatment of kikuchi disease is generally supportive. Kikuchi fujimoto disease is an idiopathic, extremely rare, more or less worldwide, and often underdiagnosed condition. The disease can have aneacute or subacute course, usually devel.
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. This is a benign and selflimited disease, frequently associated with mild fever, and occasionally with other systemic symptoms. Extranodal hematopoietic neoplasms and mimics in the head. Ideal sources for wikipedia s health content are defined in the guideline wikipedia.
It is more common in asia, but isolated cases are also reported in america, africa and europe. A rare case of kicukhi fujimoto disease histiocytic. Now he has a softball size lymph node in his abdomen that the drs think might be kikuchi disease again. 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. Extranodal involvement is uncommon, but skin rash, hepatitis, arthritis, oral ulcers. Kikuchifujimoto disease with prolonged fever in children kyungyil lee, md. Pathogenesis, diagnosis, and management of kikuchi. Kikuchifujimoto disease kfd, also called histiocytic necrotizing lymphadenitis, was initially described. Kikuchis disease took a selflimiting clinical course in most cases, but the. Disease definition of disease by medical dictionary. Bests disease an autosomal dominant inherited degeneration in which there is an accumulation of lipofuscin within the retinal pigment epithelium, which interferes with its function. To describe a clinical case of kikuchi disease with systemic features, response to ivig therapy and to.
No specific diagnostic laboratory tests are available. Kikuchi fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting, benign, and rare systemic lymphadenitis with unknown etiology. Affected patients are most often young adults under the age of 30 years. Berlins disease a traumatic phenomenon in which the posterior pole of the retina develops oedema and haemorrhages. Kikuchifujimoto disease kfd, also known as kikuchis disease or necrotizing granulomatous lymphadenitis, is rare, has a benign course and is of unknown cause. Kikuchi fujimoto disease mimicking lymphoma detected on pet. In this interesting case report, petct imaging findings of a rare kikuchifujimoto disease mimicking lymphoma in a pediatric patient. Pdf kikuchifujimoto disease with multiple extranodal. 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.
The disease frequently mimics tuberculous lymphadenitis, malign lymphoma, and many other benign and malignant conditions. 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%. 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. Management of kikuchifujimoto disease using glucocorticoid.
Pathogenesis, diagnosis, and management of kikuchifujimoto. Clinical outcome and predictive factors of recurrence among. Steroid kikuchifujimoto disease with prolonged fever in children. Kikuchi disease is a rare but benign, selflimiting disease that typically presents with prolonged fever and cervical lymphadenopathy. Kikuchi fujimoto disease mimicking lymphoma detected on. Kikuchifujimoto disease kfd, also known as kikuchi disease, is a rare lymphohistiocytic disorder first described in 1972.
The use of corticosteroids, such as prednisone, has been recommended in severe extranodal or generalized kikuchi disease. Persistent cervical lymphadenitis in a patient with prior. 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. Kfd has an excellent prognosis with little risk of fatality. Less frequent symptoms include weight loss, nausea, vomiting, sore throat.
Kikuchis disease is a rare nonmalignant disorder that affects the lymph nodes. Kikuchifujimoto 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 kfd was first described in japan in 1972. This disease has now been recognized in many other countries and has also been referred to as kikuchis disease, histiocytic necrotizing. Mar 11, 2018 kikuchi fujimotos disease kfh, which is seen more often in young asian women than in childhood, is a rare benign feature of petct that can be confused with malignant lymphoma due to common cervical hypermetabolic lymphadenopathy and high suv values.
Kikuchi fujimoto disease kfd was first described in japan in 1972. Antinuclear antibodies, antiphospholipid antibodies, antidsdna, and rheumatoid factor are usually negative, and may help in differentiation from 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, gender. Kikuchi s disease seems to be more prevalent in japanese and asian individuals. Kikuchi disease is a benign disease of the lymph nodes. It can involve both nodal and extranodal locations 3. Sep 25, 2018 kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchi fujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis.
Isolated cases are reported in north america, europe, asia and at least two cases new zealand. Diagnosis kikuchi fujimoto disease is generally diagnosed on the basis of an excisional biopsy of affected lymph nodes. Rosaidorfman disease, also known as sinus histiocytosis with massive lymphadenopathy or sometimes as destombesrosaidorfman disease, is a rare disorder of unknown cause that is characterized by abundant histiocytes in the lymph nodes or other locations throughout the body. The results of a wide range of laboratory studies are usually. Kikuchi disease kd, also known as kikuchifujimoto disease or histiocytic necrotizing lymphadenitis, is a selflimiting cause of cervical lymphadenopathy. Kikuchifujimoto disease kfd is a rare entity characterized by subacute necrotizing lymphadenopathy and frequently associated with fever. Choi, md, phd kikuchifujimoto disease kfd is a rare entity characterized by subacute necrotizing lymphadenopathy and frequently associated with fever. Kikuchi disease is a selflimiting illness which has symptoms which may overlap with hodgkins lymphoma leading to misdiagnosis in some patients.
Boheng li, junli yan, the phyu, shuangyi fan, taehoon chung, nurulhuda mustafa, baohong lin, lingzhi wang, pieter johan adam eichhorn, booncher goh, siokbian ng, dennis kappei, weejoo chng. 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. 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. Part of the highly regarded diagnostic pathology series, this updated volume is a visually stunning, easytouse reference covering all aspects of benign and malignant lesions of lymph node, spleen, and extranodal lymphomas. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among. He had it in his neck in february 2017 it was removed and he got better. Nonhodgkin lymphomas are most common, and hodgkin lymphoma occurs rarely. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchi fujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation. Extranodal hematopoietic neoplasms and mimics in the head and.
Kikuchifujimoto disease radiology reference article. Kikuchifujimoto disease kfd is a selflimiting disease that has an unknown cause and was first described independently in 1972 by kikuchi 1 and fujimoto et al 2 in japan. Kikuchifujimoto disease kikuchis lymphadenitis, kikuchis disease, histiocytic necrotising lymphadenitis, subacute necrotising lymphadenitis is a selflimited benign syndrome first described by kikuchi and fujimoto in 1972. Now he has a softball size lymph node in his abdomen that the drs think might be. Ct and mr findings dong gyu na, taesub chung, hong sik byun, hong dae kim, young hyeh ko, and jeong hwan yoon summary. A kikuchifujimoto disease case mimicking t cell lymphoma. Kikuchi disease symptoms and treatment verywell health. Kikuchi fujmoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is a rare lymphadenitis of unknown origin, but with an excellent prognosis. Kikuchi fujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a selflimited disease of complex and unclear etiology. Also called kikuchi fujimoto disease or histiocytic necrotizing lymphadenitis, this condition was discovered in japan in 1972 and since then has seen in other areas of the world. Jeffrey medeiros, 9780323477796, available at book depository with free delivery worldwide. 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. 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.
Extranodal kikuchi disease and kikuchi encephalitis springerlink. The trip database provides clinical publications about evidence. Kikuchi fujimoto disease kfd is a selflimited disease characterized by necrotizing lymphadenitis. A 31yearold previously healthy indian woman was admitted with high grade fever. Most cases occur sporadically in people with no family history of the condition. Kikuchis disease is a benign, selflimiting disorder, which is characterized by. Because it can cause cervical lymphadenopathy, kikuchi fujimoto disease must be included in the differential diagnosis of patients with sus. The mean age of onset is between the ages of 25 and 304 5 and female to male ratio is in the 4. Jan 24, 2017 kikuchi disease is fairly common in young people, predominantly young women, in asia.
The exact cause of the disease is not yet known, although some researchers have suggested it is an infection or autoimmune disorder. Castleman disease is a rare lymphoproliferative disorder of unknown etiology that most commonly presents as a mediastinal nodal mass or, in the extranodal form of the disease, a mass located in the mediastinum or retroperitoneum. Kikuchi disease with generalized lymph node, spleen and. This page may contain terms or definitions that are offensive or inappropriate for some readers. 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. Recognition of this entity is crucial, as mistaking this disease as lymphoma. The clinical and laboratory aspects of the patients confirmed by exci. It may affect the whole body or any of its parts, and its etiology, pathology, and prognosis may be known or unknown. Clinically and microscopically, it may mimic malignant lymphoma and other nonmalignant diseases. The most commonly affected extranodal organ is the. Sonographic features of axillary lymphadenopathy caused. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchifujimoto disease. First described in 1972, kd is increasingly recognized in the literature.
The disease was initially described in 1972, in japan, in young females affected by fever and cervical lymphadenopathy. Kikuchifujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting, benign, and rare systemic lymphadenitis with unknown etiology. The cause of this disease is not known, although infectious and autoimmune causes have been. Kikuchi first described the disease in 1972 in japan. Kikuchifujimotos disease kfh, which is seen more often in young asian women than in childhood, is a rare benign feature of petct that can be confused with malignant lymphoma due to common cervical hypermetabolic lymphadenopathy and high suv values. 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. Kikuchi disease kikuchifujimoto disease, histiocytic necrotizing lymphadenitis is a systemic illness of unkown etiology. Less frequent symptoms include weight loss, nausea, vomiting, sore. Sonographic features of axillary lymphadenopathy caused by. Kikuchifujimoto disease or histiocytic necrotizing lymphadenitis, is a rare, idiopathic, usually selflimited disease characterized by cervical lymphadenopathy and general symptoms was described for the first time in 1972 by japanese pathologists kikuchi and fujimoto independently. Extranodal involvement is uncommon, but skin rash, hepa titis, arthritis, oral.
Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation. Although cervical lymphadenitis in young women is the most familiar clinical presentation, it may take place in the etiology of fever in cases presenting with fever of unknown origin. Pdf extranodal involvement in kikuchi s disease is uncommon. A 33yearold male case admitted with fever, nausea, vomiting, weight loss, and leukopenia for one month. It is exceptionally uncommon for castleman disease to present in the extremities. Pdf aim histiocytic necrotising lymphadenitis, or kikuchifujimoto disease kfd, is a benign, selflimiting disorder. Melk mediates the stability of ezh2 through sitespecific.
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