Kikuchifujimoto disease kfd, also known as kikuchi disease, is a rare lymphohistiocytic disorder first described in 1972. 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. Antinuclear antibodies, antiphospholipid antibodies, antidsdna, and rheumatoid factor are usually negative, and may help in differentiation from systemic lupus erythematosus. 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. A rare cause of fever and lymphadenopathy article pdf available in clinical medicine insights. The trip database provides clinical publications about evidence. While the pathogenesis of kikuchi disease is unknown, the clinical presentation, course, and histologic changes suggest an immune response of t cells and histiocytes to an infectious agent. Kikuchifujimoto disease is a distinctive syndrome of necrotizing lymphadenitis which was formally described in 1972 by two japanese pathologists, kikuchi and fujimoto. Clinical examination revealed enlarged rightsided lymph nodes in the neck. Histiocytic necrotizing lymphadenitis kikuchifujimoto disease. Characteristic findings on pathology include the presence of crescenteric. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchi fujimoto disease.
Kikuchifujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence. Clinical studies of kikuchis disease full text view. Kikuchis disease of the mesenteric lymph nodes presenting. Also called histiocytic necrotizing lymphadenitis, kikuchifujimoto disease. We report a 25 yr old female who presented with fever, polyarthritis and cervical lymphadenopathy. The following cases have burn reported for clinical interest and for the rarity of. However, when it occurs in the context of connective tissue disease, particularly systemic lupus erythematosus sle, it is usually associated with a flareup of the patients symptoms, requiring treatment, and. 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. Kikuchifujimoto disease kfd is an extremely rare disease with a worldwide distribution and higher prevalence in asians. Department of pathology, college of medicine, hanyang university, 17 haengdangdong, seongdonggu, seoul 3792, korea tel. Complete blood cell cbc count findings include the following. A 12yearold female suffered from cervical lymphadenopathy, leukocytopenia, fever and.
For language access assistance, contact the ncats public information officer. Kikuchifujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare benign, selflimiting cervical lymphadenitis of unknown etiology. It is rare in caucasians, normally occurring in those of asian descent. Kikuchi disease is characterized histopathologically by. 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. If you have problems viewing pdf files, download the latest version of adobe reader. Kikuchi disease is an idiopathic, generally selflimiting cause for lymphadenitis that can be clinically and histologically mistaken for lymphoma or systemic lupus erythematosus. Kimuras disease is a lymphadenopathy that usually involves the head and neck region with a predilection for young adult asian men. It was more common in asian people, especially in japanese. They described the disease as lymphadenitis with focal proliferation of reticular cells accompanied by. Study design this was a retrospective study of children diagnosed with kikuchifujimoto disease from march 2003 to february 2015 in south korea.
Initially described in japan and other asian countries. Pathogenesis, diagnosis, and management of kikuchi. 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. Histologically, the dermatological manifestations of kikuchifujimoto disease have features in common with systemic lupus erythematosus.
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. This means that kikuchi disease, or a subtype of kikuchi disease, affects less than 200,000 people in the us population. The aetiology is poorly understood, but it appears to be an autoimmune disorder with a possible infectious trigger. Ferry, in diagnostic pathology of infectious disease second edition, 2018. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting benign lymphadenopathy of unknown origin that predominantly affects young adults. They described the disease as lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris.
Kikuchis disease is a selflimited necrotizing lymphadenitis that is characterized by cervical lymphadenopathy and fever. It is a benign, selflimited disease that predominantly occurs in young women. Successful treatment of severe kikuchis disease with. Cat scratch disease csd is usually a selflimited infection caused by a curved gramnegative, bacteria bartonella henselae. Pathology of kikuchifujimoto disease top ten facts dr. Between 1990 and 2002, 96 patients 68 women, 28 men. First described independently by kikuchi 27 and fujimoto 19 in 1972, kikuchifujimoto disease is increasingly recognized in the literature. American journal of clinical pathology, volume 1, issue 2. Introduction kikuchis disease kd is a histiocytic necrotizing lymphadenitis that was described for the first time in japan in 1972 almost simultaneously by kikuchi and fujimoto12. Kikuchi disease, is a rare pathology of the lymph nodes.
Kikuchi first described the disease in 1972 in japan. Ultrasonographic differentiation between kikuchis disease. The bacteria form filaments up to 10 micrometer or longer. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, kikuchi lymphadenitis, kikuchifujimoto disease, and. 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. Bdca2, a novel plasmacytoid dendritic cellspecific type ii ctype lectin. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Examples of singleuser license include conferences, seminars, presentations, and onetime teaching courses. Kikuchifujimoto disease, also known as kikuchis disease or histiocytic necrotizing lymphadenitis, is a benign, selflimiting cause of lymphadenopathy, variably associated with systemic symptoms. Kikuchi disease symptoms and treatment verywell health. Kikuchi fujimoto disease kfd was first described in japan in 1972 almost simultaneously by kikuchi and fujimoto. Pathology of cat scratch disease dr sampurna roy md. Misdiagnosing kfd as lymphoma or systemic lupus erythematosus is not uncommon due to the similarity of clinical and histopathological features of these diseases.
Less frequent symptoms include weight loss, nausea, vomiting, sore throat. 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. Histiocytic necrotizing lymphadenitis, the socalled kikuchifujimoto disease, was first described in 1972 by two independent japanese pathologists, kikuchi and fujimoto 12 3 is a rare. It is a benign and selflimiting disorder, characterized by regional cervical lymphadenopathy accompanied with mild fever and night sweats. A reappraisal using novel immunohistochemistry combinations. We characterized the clinical and ct findings in a large group of patients with kd. Kikuchifujimoto disease radiology reference article. Jeffrey medeiros, in diagnostic surgical pathology of the head and neck second edition, 2009. Kikuchi disease is a selflimiting illness which has symptoms which may overlap with hodgkins lymphoma leading to misdiagnosis in some patients. Kikuchifujimoto disease, is usually a benign selflimiting disease which typically affects young females under the age of 30 years and resolves without treatment within six months. It has a low recurrence rate of 3% to 4%, 1 3 and only several fatal cases have been reported.
Lactate dehydrogenase and alanine aminotransferase concentrations can be raised. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchifujimoto disease, is an uncommon cause of lymphadenopathy. Kikuchi fujimoto disease is a selflimited disease that usually resolves within a few months. Kikuchi disease symptoms, diagnosis, treatments and causes. Fineneedle aspiration cytology of kikuchi fujimoto disease. The clinical features are such that it is often mistaken for infectious. It predominantly affects young women and can closely mimic infective and immunological disorders. Kikuchis disease, lymphadenitis, lymphoma, systemic lupus erythematosus. Abstract kikuchifujimoto disease, or histiocytic necrotizing lymphadenitis, is a. Electronic medical records were searched for clinical and laboratory manifestations. Differentiating this disease from common lymphatic disorder is extremely important from the pathologists point of view, which is highlighted in the article. Novel markers of normal and neoplastic human plasmacytoid dendritic cells. Fineneedle aspiration and excision biopsy were undertaken.
The exact cause of the disease is not yet known, although some researchers have suggested it is an infection or autoimmune disorder. This disease is of unknown etiology, but an infectious etiology has been implicated in the. Kikuchifujimoto disease also known as kikuchi disease or histiocytic necrotizing. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation. Results among 86 histopathologically confirmed cases, the mean age was. Histopathology of the involved lymph nodes differentiates kikuchi disease from several more serious conditions that it may mimic. Kikuchis disease of the mesenteric lymph nodes presenting as acute appendicitis corresponding author seung sam paik, m. Kikuchifujimoto disease kfd is a histiocytic necrotising lymphadenitis, which is a benign disease of unknown aetiology.
The disease was described in 1972 by kikuchi 1 and independently by fujimoto et al 2. Kikuchifujimoto disease with lymph node, spleen, and. Histiocytic necrotizing lymphadenitis hnl is a rare benign disorder characterized. Kikuchifujimoto disease kfd is typically a selflimited, benign illness which presents with fever and lymphadenopathy. Histopathology of the involved lymph node differentiate kikuchis disease from several more serious conditions particularly lymphomas, which it may mimic1. Furthermore, signs and symptoms of kikuchi disease may vary on an individual basis for each patient. Kikuchi fujimoto disease also known as histiocytic necrotizing lymphadenitis is an idiopathic benign self limiting condition with a favourable outcome, usually affecting young women. Kikuchi disease genetic and rare diseases information. It is a rare, self limiting, benign form of histiocytic necrotizing lymphadenitis, which can be mistaken for tuberculosis, lymphoma or systemic lupus. Kikuchis disease is an idiopathic illness characterized by a selflimiting lymphadenitis that normally resolves over subsequent weeks or months without specific treatment. 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. Find the top 100 most popular items in amazon books best sellers. Kd is a selflimited necrotizing lymphadenitis and occurs predominantly in females between 20 and 30 years old. The condition was first described in 1972 by masahiro kikuchi 19342012, a professor of histopathology and haematopathology, at fukuoka university medical.
It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchifujimoto disease. We report a case of kfd in a 47 yearold man who presented with weight loss, fever, enlarged lymph nodes and elevated serum levels. It is easily seen in tissue sections of the skin, lymph nodes, and conjunctiva, when stained by a silver impregnation technique. Further radiological scans showed a mass near the carotid and enlarged level v 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. Examples of multiuser license include books, book chapters, educational material, and other publications with multiple copies. Ideal sources for wikipedia s health content are defined in the guideline wikipedia. We provide 1 original jpeg or tiff and 1 highquality optimized image without our logo. This signs and symptoms information for kikuchi disease has been gathered from various sources, may not be fully accurate, and may not be the full list of kikuchi disease signs or kikuchi disease symptoms. The disease is characterised by a unilateral posterior cervical lymphadenopathy. 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%.
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