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. Fujimoto and colleagues independently described kikuchi s disease in the same year. Pathology of kikuchifujimoto disease top ten facts dr. Kikuchifujimoto or kikuchi lymphadenopathy is a rare, regional, selflimited, necrotizing, histiocytic lymphadenitis that is frequently associated with mild fever and other systemic symptoms. It is rare in caucasians, normally occurring in those of asian descent. The aetiology is poorly understood, but it appears to be an autoimmune disorder with a possible infectious trigger. Differentiation from tuberculous lymphadenitis based on patterns of nodal necrosis on ct. It is a selflimiting, rare, necrotizing form of lymphadeni tis that most commonly affects young women. Kikuchifujimoto disease kfd was first reported in 1972 almost simultaneously by kikuchi and fujimoto and associates. Kikuchi first described the disease in 1972 in japan. Kikuchis disease nord national organization for rare.
Jun 17, 2016 kikuchi fujimoto disease kfd is an extremely rare disease with a worldwide distribution and higher prevalence in asians. Examples of multiuser license include books, book chapters, educational material, and other publications with multiple copies. Also called histiocytic necrotizing lymphadenitis, kikuchi fujimoto disease initially described in japan and other asian countries. To our knowledge, there is no previous study comparing the clinical and laboratory characteristics of patients from different geographical parts of the world. To date only eight cases have been reported from nepal. Kikuchifujimoto disease is so named because kikuchi and fujimoto were the first scientists to describe it in japan in 1972. 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. Fujimoto and colleagues independently described kikuchi disease in the same year. Cervical lymphadenopathy secondary to kikuchifujimoto. While most common in southeast asia, where kfd was initially described in 1972 in young women, this lesion has since been.
Serology was negative for toxoplasmosis, ebv or catscratch disease. The clinical, histopathological and immunohistochemical features appear to point to a viral etiology, a hypothesis that still has not been proven. Also called histiocytic necrotizing lymphadenitis, kikuchifujimoto disease initially described in japan and other asian countries. She was eventually diagnosed as having the kikuchi fujimoto disease and. Kikuchi fujimoto disease kfd or histiocytic necrotizing lymphadenitis is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Sep 14, 2016 kikuchi disease is a benign noncancerous condition of the lymph nodes. Kikuchifujimoto disease radiology reference article. Kikuchifujimoto disease orphanet journal of rare diseases. In 1972, kikuchi and fujimoto independently described this entity. Kikuchi fujimoto disease kfd is typically a selflimited, benign illness which presents with fever and lymphadenopathy. Kikuchi fujimoto disease associated with cryptogenic organizing pneumonia. Kikuchi disease is characterized histopathologically by.
Kikuchi fujimoto disease in patient with systemic phacomatosis pigmentovascularis. Enigmatic kikuchifujimoto disease american journal of. Kikuchi disease is a benign noncancerous condition of 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. Kikuchi disease genetic and rare diseases information. Involvement of intraabdominal lymph nodes has been rarely reported. An uncommon presentation of kikuchifujimoto disease as.
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. We present 41yearold nigerian woman who was investigated extensively for unilateral left cervical lymphadenopathy. Research for kylees kikuchifujimoto disease facebook. Kikuchifujimoto disease, or histiocytic necrotizing lymphadenitis, is a selflimited condition, characterized by benign lymphadenopathy with associated fevers and systemic symptoms. Kikuchifujimoto disease, or histiocytic necrotizing lymphadenitis, is a relatively new disease entity seen in the u. 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%. 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. Also known as histiocytic necrotizing lymphadenitis, kfd is usually considered a benign, self. Jun 01, 2008 with kikuchi fujimoto disease considered as part of the differential diagnosis, otolaryngologists can prompt pathologists to look for the features specific to this disease process. Recognition of this condition is crucial, especially because it can easily be mistaken for tuberculosis, lymphoma, or even adenocarcinoma. Gaman m, vladareanu am, dobrea c, onisai m, marinescu c, voican i, et al.
Kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among. It occurs worldwide with a higher prevalence among asians and women below the age of forty years. Examples of singleuser license include conferences, seminars, presentations, and onetime teaching courses. Kikuchifujimoto disease is an uncommon disorder with worldwide distribution, characterized by fever and benign enlargement of the lymph nodes, primarily affecting young adults. Less frequent symptoms include weight loss, nausea, vomiting, sore throat. 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. Kikuchi disease, is a rare pathology of the lymph nodes. Although the disease has been reported from all over the world and more so from asia, it is rare. 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. We provide 1 original jpeg or tiff and 1 highquality optimized image without our logo. Awareness about this disorder may help prevent misdiagnosis and inappropriate investigations and treatment.
They described the disease as lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris. Kikuchi fujimoto disease also known as histiocytic necrotizing lymphadenitis is an idiopathic benign self limiting condition with a favourable outcome, usually affecting young women. The disease frequently mimics tuberculous lymphadenitis, malign lymphoma, and many other benign and malignant conditions. We experienced an uncommon presentation of kikuchi fujimoto disease kfd with sole mediastinal lymphadenopathy in senior age, which was histologically diagnosed by thoracoscopic biopsy leading to appropriate therapy. While the exact cause of this condition is unknown, infectious and autoimmune causes have been suggested. Cervical lymphadenopathy, fever and raised erythrocyte sedimentation rate are usual presenting features of this. 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. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchi fujimoto disease, is an uncommon cause of lymphadenopathy. Kikuchifujimoto disease kfd, also known as kikuchi disease, is a rare lymphohistiocytic disorder first described in 1972. Kikuchi fujimoto s disease kfd, histiocytic necrotizing lymphadenitis, is a rare selflimiting condition characterized by lymphadenopathy, fever and neutropenia.
Pathogenesis, diagnosis, and management of kikuchifujimoto. An uncommon presentation of kikuchi fujimoto disease. It most commonly affects adults younger than 40 years of age and of asian descent. Causative agents of kikuchi fujimoto disease histiocytic necrotizing lymphadenitis. Kikuchifujimoto disease kfd, a lesser known cause of a selflimiting neck mass that is often confused with malignant lymphoma, is unfam. Kylee davis a sixth grader at conyers middle school in conyers, ga. The disease was described in 1972 by kikuchi 1 and independently by fujimoto et al 2. Early biopsy and diagnosis can help minimize unnecessary testing and potentially harmful treatments. 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. A 69yearold man was admitted due to intermittent high fever, general malaise, and appetite loss lasting over 3. Kikuchi fujimoto disease kfd is a benign, selflimiting disease characterized by histiocytic necrotising lymphadenitis. Less common symptoms include weight loss, nausea, vomiting, and sore throat. Kikuchi fujimoto disease, or histiocytic necrotizing lymphadenitis, is a relatively new disease entity seen in the u.
Kikuchifujimoto disease is a rare, selflimiting disease of unknown etiology, manifested by lymphadenopathy, fever and less specific symptoms such as. 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. Although the cause is unknown, infectious and autoimmune etiologies have been proposed for this disease. Kfd has a worldwide distribution, and asiatic people have a higher prevalence. Histiocytic necrotizing lymphadenitis kikuchis disease. Kikuchifujimoto disease kfd was first described in japan in 1972. Overlap between systemic lupus erythematosus and kikuchi fujimoto disease. 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. The following cases have burn reported for clinical interest and for the rarity of the reports about this condition in ent literature.
Successful treatment of severe kikuchis disease with. 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. Kikuchifujimoto disease kfd is a rare entity characterized by subacute necrotizing lymphadenopathy and frequently associated with fever. Kikuchifujimotos disease kfd, histiocytic necrotizing lymphadenitis, is a rare selflimiting condition characterized by lymphadenopathy, fever and neutropenia. The kikuchi fujimoto is a rare, selflimiting disease, which is characterized by regional lymphadenopathy.
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