Jun 17, 2016 kikuchi fujimoto disease kfd is an extremely rare disease with a worldwide distribution and higher prevalence in asians. It occurs worldwide with a higher prevalence among asians and women below the age of forty years. The following cases have burn reported for clinical interest and for the rarity of. Sep 14, 2016 kikuchi disease is a benign noncancerous condition of the lymph nodes.
Kikuchifujimoto disease previous article addressing invisibility, inferiority, and powerlessness to achieve gains in maternal health for ultrapoor women in february, 20, a 30yearold ugandanborn woman, working in the uk, presented to the accident and emergency department with a 4week history of arthralgia, fevers, and lethargy, and a. Kikuchis disease usually presents as solitary or multiple enlarged cervical lymph nodes and only rarely with generalized lymphadenopathy. Kikuchis disease of the mesenteric lymph nodes presenting as. 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 report a case of kfd in a 47 yearold man who presented with weight loss, fever, enlarged lymph nodes and elevated serum levels. 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 kfd or histiocytic necrotizing lymphadenitis is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Histiocytic necrotizing lymphadenitis hnl is a rare benign disorder. Kikuchis disease, lymphadenitis, lymphoma, 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%. Kikuchi fujimoto disease kfd, a rare clinicopathological entity, is a benign and selflimiting disease. Less common symptoms include weight loss, nausea, vomiting, and sore throat. Kikuchi disease, is a rare pathology of the lymph nodes.
Antinuclear antibodies, antiphospholipid antibodies, antidsdna, and rheumatoid factor are usually negative, and may help in differentiation from systemic lupus erythematosus. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting benign lymphadenopathy of unknown origin that predominantly affects young adults. Fineneedle aspiration cytology of kikuchi fujimoto disease. Kfd can occur in association with systemic lupus erythematosus. Kikuchi disease genetic and rare diseases information.
Fortyfive examples of kikuchis lymphadenitis kl, 5 kikuchilike lupus. 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. It predominantly affects young women and can closely mimic infective and immunological disorders. Kikuchi fujimoto disease is an extremely rare, benign, and selflimiting disease that. 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. Kikuchifujimoto disease radiology reference article.
The kikuchifujimoto is a rare, selflimiting disease, which is characterized by regional lymphadenopathy. The clinical features are such that it is often mistaken for infectious. For language access assistance, contact the ncats public information officer. 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.
Reprints and eprints sponsored supplements branded books. Kikuchifujimoto disease kfd is an extremely rare disease with a worldwide distribution and higher prevalence in asians. Kikuchi disease is a benign noncancerous condition of the lymph nodes. This means that kikuchi disease, or a subtype of kikuchi disease, affects less than 200,000 people in the us population. 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. Kikuchis disease is an idiopathic illness characterized by a selflimiting lymphadenitis that normally resolves over subsequent weeks or months without specific treatment. Kikuchi fujimoto 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 is a rare cause of lymphadenopathy, commonly seen in individuals of asian descent and frequently associated with fever and, sometimes. While most common in southeast asia, where kfd was initially described in 1972 in young women, this lesion has since been. Initially, bacterial agents, such as yersinia, bartonella and protozoan toxoplasma were thought to cause the disease, recently atypical mycobacterium szulgi has been isolated from thailand, brucella has also. Myeloperoxidase expression by histiocytes in kikuchis and kikuchi. Pathology, university of michigan, 5230 medical sciences bldg 1, 1 catherine st, ann arbor, mi, 48109 email.
It was first described in 1972 by kikuchi and fujimoto in japan independently. Two cases are reported in the japanese literature 1 and one from india. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation. The disease frequently mimics tuberculous lymphadenitis, malign lymphoma, and many other benign and malignant conditions. Kikuchi disease symptoms, diagnosis, treatments and causes. 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. Kikuchifujimoto disease with lymph node, spleen, and. Kikuchi disease is an uncommon, benign, rarely fatal, disease, reported independently, almost simultaneously, by kikuchi and fujimoto et al. International classification of diseases for oncology. This disorder is often mistaken for malignant lymphoma, especially cervical adenopathy because the symptoms are very similar. Department of pathology, college of medicine, hanyang university, 17 haengdangdong, seongdonggu, seoul 3792, korea tel. He presented with fevers, weight loss and tender cervical lymph nodes.
The trip database provides clinical publications about evidence. Management of kikuchifujimoto disease using glucocorticoid. Kikuchi fujimoto disease also known as histiocytic necrotizing lymphadenitis is an idiopathic benign self limiting condition with a favourable outcome, usually affecting young women. Source national institutes of health nih ophanet, a consortium of european partners, currently defines a condition rare when it affects 1 person per 2,000. The illness in may 1999 was similar to the present one and so this case represents a case of recurrent kikuchi fujimoto. To our knowledge, there is no previous study comparing the clinical and laboratory characteristics of patients from different geographical parts of the world. Kfd has a worldwide distribution, and asiatic people have a higher prevalence. Less frequent symptoms include weight loss, nausea, vomiting, sore throat. Kikuchi disease symptoms and treatment verywell health. This disease is of unknown etiology, but an infectious etiology has been implicated in the.
Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchifujimoto disease, is an uncommon cause of lymphadenopathy. We characterized the clinical and ct findings in a large group of patients with kd. Pathogenesis, diagnosis, and management of kikuchi. Moreover, such observations indicate that patients with kikuchis disease should be kept under observation for several years to ensure that they are not at risk for the development of systemic lupus erythematosus. Kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among. 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 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. 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. She was eventually diagnosed as having the kikuchifujimoto disease and. 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 is a selflimited necrotizing lymphadenitis that is characterized by cervical lymphadenopathy and fever. Kikuchis disease is a selflimited necrotizing lymphadenitis that is characterized by cervical lymphadenopathy and fever. Jeffrey medeiros, in diagnostic surgical pathology of the head and neck second edition, 2009.
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. 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. Between 1990 and 2002, 96 patients 68 women, 28 men. 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. The initial description was referred as kikuchis and fujimoto disease or. Successful treatment of severe kikuchis disease with. Kikuchifujimoto disease kfd, also known as kikuchi disease, is a rare lymphohistiocytic disorder first described in 1972.
Kikuchis disease of the mesenteric lymph nodes presenting as acute appendicitis corresponding author seung sam paik, m. This book is distributed under the terms of the creative commons attribution 4. It is a rare, self limiting, benign form of histiocytic necrotizing lymphadenitis, which can be mistaken for tuberculosis, lymphoma or systemic lupus. Kikuchis disease of the mesenteric lymph nodes presenting. A case of a 55 year old gentleman is described here. Complete blood cell cbc count findings include the following. In patients with kikuchi disease, diagnostic laboratory and radiologic test findings are nonspecific. 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. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchifujimoto disease. Kikuchifujimoto disease was diagnosed after cervical lymph node biopsy. It is a benign and selflimiting disorder, characterized by regional cervical lymphadenopathy accompanied with mild fever and night sweats. Kikuchifujimoto disease is a rare benign, condition of necrotising histiocytic lymphadenitis. 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 kfd, or histiocytic necrotizing lymphadenitis, is a benign and selflimited disease that mainly affects young women. Ideal sources for wikipedia s health content are defined in the guideline wikipedia. Mar 22, 2014 kikuchi fujimoto disease previous article addressing invisibility, inferiority, and powerlessness to achieve gains in maternal health for ultrapoor women in february, 20, a 30yearold ugandanborn woman, working in the uk, presented to the accident and emergency department with a 4week history of arthralgia, fevers, and lethargy, and a. Recognizing a common presentation of an uncommon condition ramya punati, md thomas jefferson university, ramya. 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. A benign, rare disorder that affects young women more so than men with recurrent necrotizing lymphadenitis often associated with other systemic inflammatory or infectious disorders. Covering all aspects of skin disease from basic science through pathology and epidemiology to clinical practice, the text is recognized for its unparalleled coverage of diagnosis. Kikuchi fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting, benign, and rare systemic lymphadenitis with unknown etiology.
Kikuchi disease kd also known as kikuchifujimoto disease, necrotizing histiocytic lymphadenopathy is an uncommon, benign, enigmatous, selflimiting, rarely fatal, disease of young caucasian females, presenting with firm, tender, unilateral cervical lymphadenopathy. 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. Pdf kikuchifujimoto disease in a 30yearold caucasian female. Kikuchi disease kd also known as kikuchi fujimoto disease, necrotizing histiocytic lymphadenopathy is an uncommon, benign, enigmatous, selflimiting, rarely fatal, disease of young caucasian females, presenting with firm, tender, unilateral cervical lymphadenopathy. Symptomatic treatment was provided and an uneventful full recovery was made. Ad department of pathology, stanford university medical center, ca 94305.
Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Here are links to possibly useful sources of information about kikuchi disease. The medicine forum volume 17 article 17 2016 kikuchi disease. Medline abstract for reference 15 of kikuchi disease. The cardinal symptoms are fever, lymphadenopathy and night sweat.
Kikuchifujimoto disease rooks textbook of dermatology. Mar 15, 2006 kikuchifujimoto disease kfd was first described in japan in 1972. Kuo, kikuchis disease histiocytic necrotizing lymphadenitis. The disease was described in 1972 by kikuchi 1 and independently by fujimoto et al 2. Kikuchi disease is characterized histopathologically by. We recommend sharing personal contact information by private. The exact cause of the disease is not yet known, although some researchers have suggested it is an infection or autoimmune disorder. Kikuchifujimoto disease kfd also known as histiocytic necrotizing. Lymph node biopsies show histiocytic necrotizing lymphadenitis, fragmentation, necrosis. Histiocytic necrotizing lymphadenitis, or kikuchis disease, is a selflimiting cervical lymphadenitis of unknown origin. You probably already know most of the information, but thought it might be of interest to you.
We present 41yearold nigerian woman who was investigated extensively for unilateral left cervical lymphadenopathy. The main symptoms include swollen lymph nodes in the neck, mild fever, and night sweats. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Kikuchifujimoto disease kfd was first described in japan in 1972.
The following cases have burn reported for clinical interest and for the rarity of the reports about this condition in ent literature. While the exact cause of this condition is unknown, infectious and autoimmune causes have been suggested. Pdf kikuchifujimoto disease is a rare, selflimited, histiocytic, necrotizing lymphadenitis first. Pathogenesis, diagnosis, and management of kikuchifujimoto. Kikuchifujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence. We report a 25 yr old female who presented with fever, polyarthritis and cervical lymphadenopathy.
Sep 25, 2018 in patients with kikuchi disease, diagnostic laboratory and radiologic test findings are nonspecific. This association is not well defined, but some authors recommend following up patients clinically for evidence of autoimmune disease. Kikuchi first described the disease in 1972 in japan. Kikuchifujimoto disease, histiocytic necrotizing lymphadenitis. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchi fujimoto disease. Patients present with localized lymphadenopathy, fever, and leukopenia in up to half of the cases. Management of kikuchis disease using glucocorticoid the. Kikuchis disease nord national organization for rare. 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. Kikuchifujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare benign, selflimiting cervical lymphadenitis of unknown etiology. Kikuchi disease is a selflimiting illness which has symptoms which may overlap with hodgkins lymphoma leading to misdiagnosis in some patients. Kikuchifujimoto disease orphanet journal of rare diseases.
Fujimoto and colleagues independently described kikuchis disease in the same year. Enigmatic kikuchifujimoto disease american journal of. Kikuchifujimoto disease kfd is typically a selflimited, benign illness which presents with fever and lymphadenopathy. Ferry, in diagnostic pathology of infectious disease second edition, 2018. We want to help promote all of your pathology related books. If you have problems viewing pdf files, download the latest version of adobe reader. The aetiology is poorly understood, but it appears to be an autoimmune disorder with a possible infectious trigger. Kikuchifujimoto disease kfd, a rare clinicopathological entity, is a benign and selflimiting disease.
Kimuras disease is a lymphadenopathy that usually involves the head and neck region with a predilection for young adult asian men. Kikuchifujimoto disease kimura disease leukaemia cutis lymphomatous infiltration of the skin. Kikuchi fujimoto disease kfd was first described in japan in 1972 almost simultaneously by kikuchi and fujimoto. 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, also called histiocytic necrotizing lymphadenitis or kikuchi fujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation. Aseptic meningitis in kikuchi fujimoto disease is described in the literature but is rare. It most commonly affects asian adult females younger than 40 years of age. Histology of the affected lymph nodes revealed necrotizing lymphadenitis, consistent with kikuchifujimoto disease. Sep 25, 2018 kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchi fujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. It is rare in caucasians, normally occurring in those of asian descent. Arch pathol lab medvol 142, november 2018 kikuchi fujimoto disease. American journal of clinical pathology, volume 1, issue 2. There are reported associations between systemic lupus erythematosus sle and kikuchis disease.
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