Kikuchifujimoto disease kfd is a histiocytic necrotising lymphadenitis, which is a benign disease of unknown aetiology. Ideal sources for wikipedia s health content are defined in the guideline wikipedia. The disease is characterised by a unilateral posterior cervical lymphadenopathy. Between 1990 and 2002, 96 patients 68 women, 28 men. The disease was described in 1972 by kikuchi 1 and independently by fujimoto et al 2. Lactate dehydrogenase and alanine aminotransferase concentrations can be raised. Kikuchifujimoto disease kfd is typically a selflimited, benign illness which presents with fever and lymphadenopathy. It is easily seen in tissue sections of the skin, lymph nodes, and conjunctiva, when stained by a silver impregnation technique. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Kikuchifujimoto disease also known as kikuchi disease or histiocytic necrotizing. Pathogenesis, diagnosis, and management of kikuchi. If you have problems viewing pdf files, download the latest version of adobe reader. Less frequent symptoms include weight loss, nausea, vomiting, sore throat. They described the disease as lymphadenitis with focal proliferation of reticular cells accompanied by.
Kikuchi disease genetic and rare diseases information. Fineneedle aspiration cytology of kikuchi fujimoto disease. Examples of singleuser license include conferences, seminars, presentations, and onetime teaching courses. They described the disease as lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris. Histopathology of the involved lymph nodes differentiates kikuchi disease from several more serious conditions that it may mimic. We characterized the clinical and ct findings in a large group of patients with kd. Clinical examination revealed enlarged rightsided lymph nodes in the neck. We want to help promote all of your pathology related books. First described independently by kikuchi 27 and fujimoto 19 in 1972, kikuchifujimoto disease is increasingly recognized in the literature. 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 a benign, selflimited disease that predominantly occurs in young women.
Examples of multiuser license include books, book chapters, educational material, and other publications with multiple copies. Histiocytic necrotizing lymphadenitis kikuchifujimoto disease. American journal of clinical pathology, volume 1, issue 2. Kikuchi first described the disease in 1972 in japan. 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 disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchifujimoto disease, is an uncommon cause of lymphadenopathy.
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. Kimuras disease is a lymphadenopathy that usually involves the head and neck region with a predilection for young adult asian men. The following cases have burn reported for clinical interest and for the rarity of. Furthermore, signs and symptoms of kikuchi disease may vary on an individual basis for each patient. Bdca2, a novel plasmacytoid dendritic cellspecific type ii ctype lectin. Further radiological scans showed a mass near the carotid and enlarged level v lymph nodes.
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. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting benign lymphadenopathy of unknown origin that predominantly affects young adults. The clinical features are such that it is often mistaken for infectious. 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. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchifujimoto disease. Misdiagnosing kfd as lymphoma or systemic lupus erythematosus is not uncommon due to the similarity of clinical and histopathological features of these diseases. It was more common in asian people, especially in japanese. 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.
Ferry, in diagnostic pathology of infectious disease second edition, 2018. Pathology of kikuchifujimoto disease top ten facts dr. 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. Kikuchis disease is a selflimited necrotizing lymphadenitis that is characterized by cervical lymphadenopathy and fever. Kd is a selflimited necrotizing lymphadenitis and occurs predominantly in females between 20 and 30 years old. Kikuchis disease is an idiopathic illness characterized by a selflimiting lymphadenitis that normally resolves over subsequent weeks or months without specific treatment. Antinuclear antibodies, antiphospholipid antibodies, antidsdna, and rheumatoid factor are usually negative, and may help in differentiation from systemic lupus erythematosus. Histiocytic necrotizing lymphadenitis hnl is a rare benign disorder characterized. 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. Kikuchi fujimoto disease is a selflimited disease that usually resolves within a few months. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is a disease that affects the lymph nodes, causing lymph node inflammation. Kikuchifujimoto disease kfd, also known as kikuchi disease, is a rare lymphohistiocytic disorder first described in 1972. For language access assistance, contact the ncats public information officer.
Also called histiocytic necrotizing lymphadenitis, kikuchifujimoto disease. Study design this was a retrospective study of children diagnosed with kikuchifujimoto disease from march 2003 to february 2015 in south korea. Kikuchifujimoto disease is a distinctive syndrome of necrotizing lymphadenitis which was formally described in 1972 by two japanese pathologists, kikuchi and fujimoto. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchi fujimoto disease. It is also known as histiocytic necrotising lymphadenitis, abbreviated hnl, and kikuchifujimoto disease. Initially described in japan and other asian countries. Kikuchis disease of the mesenteric lymph nodes presenting. The condition was first described in 1972 by masahiro kikuchi 19342012, a professor of histopathology and haematopathology, at fukuoka university medical. Pathology of cat scratch disease dr sampurna roy md. In patients with kikuchi disease, diagnostic laboratory and radiologic test findings are nonspecific. Ultrasonographic differentiation between kikuchis disease. Histopathology of the involved lymph node differentiate kikuchis disease from several more serious conditions particularly lymphomas, which it may mimic1.
Histologically, the dermatological manifestations of kikuchifujimoto disease have features in common with systemic lupus erythematosus. Differentiating this disease from common lymphatic disorder is extremely important from the pathologists point of view, which is highlighted in the article. The trip database provides clinical publications about evidence. Electronic medical records were searched for clinical and laboratory manifestations. Cat scratch disease csd is usually a selflimited infection caused by a curved gramnegative, bacteria bartonella henselae. Kikuchifujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence.
It is a benign and selflimiting disorder, characterized by regional cervical lymphadenopathy accompanied with mild fever and night sweats. Complete blood cell cbc count findings include the following. Jeffrey medeiros, in diagnostic surgical pathology of the head and neck second edition, 2009. Results among 86 histopathologically confirmed cases, the mean age was. We provide 1 original jpeg or tiff and 1 highquality optimized image without our logo. 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, also known as kikuchis disease or histiocytic necrotizing lymphadenitis, is a benign, selflimiting cause of lymphadenopathy, variably associated with systemic symptoms. It is a rare, self limiting, benign form of histiocytic necrotizing lymphadenitis, which can be mistaken for tuberculosis, lymphoma or systemic lupus. A 12yearold female suffered from cervical lymphadenopathy, leukocytopenia, fever and.
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. Characteristic findings on pathology include the presence of crescenteric. Fineneedle aspiration and excision biopsy were undertaken. Kikuchi disease symptoms and treatment verywell health. Kikuchi disease symptoms, diagnosis, treatments and causes. 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 rare in caucasians, normally occurring in those of asian descent. 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. Kikuchi fujimoto disease also known as histiocytic necrotizing lymphadenitis is an idiopathic benign self limiting condition with a favourable outcome, usually affecting young women. Abstract kikuchifujimoto disease, or histiocytic necrotizing lymphadenitis, is a. Kikuchifujimoto disease kfd is an extremely rare disease with a worldwide distribution and higher prevalence in asians. A reappraisal using novel immunohistochemistry combinations. Kikuchi disease, is a rare pathology of the lymph nodes. Find the top 100 most popular items in amazon books best sellers.
Clinical studies of kikuchis disease full text view. Kikuchis disease of the mesenteric lymph nodes presenting as acute appendicitis corresponding author seung sam paik, m. The exact cause of the disease is not yet known, although some researchers have suggested it is an infection or autoimmune disorder. Department of pathology, college of medicine, hanyang university, 17 haengdangdong, seongdonggu, seoul 3792, korea tel. Kikuchi disease is a benign noncancerous condition of the lymph nodes.
Kikuchifujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare benign, selflimiting cervical lymphadenitis of unknown etiology. Novel markers of normal and neoplastic human plasmacytoid dendritic cells. Kikuchi disease is characterized histopathologically by. It has a low recurrence rate of 3% to 4%, 1 3 and only several fatal cases have been reported. 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, is usually a benign selflimiting disease which typically affects young females under the age of 30 years and resolves without treatment within six months. The aetiology is poorly understood, but it appears to be an autoimmune disorder with a possible infectious trigger. Kikuchifujimoto disease, also known as subacute necrotizing lymphadenitis or subacute necrotizing histiocytosis, is an idiopathic disease characterized usually by cervical lymph node enlargement 80%. 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.
This disease is of unknown etiology, but an infectious etiology has been implicated in the. Kikuchifujimoto disease radiology reference article. 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. We report a 25 yr old female who presented with fever, polyarthritis and cervical lymphadenopathy. 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. This means that kikuchi disease, or a subtype of kikuchi disease, affects less than 200,000 people in the us population. Successful treatment of severe kikuchis disease with.
Kikuchifujimoto disease with lymph node, spleen, and. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchifujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. A rare cause of fever and lymphadenopathy article pdf available in clinical medicine insights. Kikuchi fujimoto disease kfd was first described in japan in 1972 almost simultaneously by kikuchi and fujimoto. 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.
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