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