Acute onset tender cervical lymphadenopathy, fever and night sweats with little evidence of infection should raise suspicion of kfd among clinicians. Kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among. Kikuchifujimoto disease kfd is a rare entity charac terized by subacute necrotizing lymphadenopathy and frequently associated with fever. Case report of kikuchifujimoto disease from subsaharan. Kikuchi fujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is a relatively rare condition characterized by subacute necrotizing regional lymphadenopathy. Kikuchifujimoto disease orphanet journal of rare diseases. It is also known as histiocytic necrotising lymphadenitis, 1 abbreviated hnl, and kikuchi fujimoto disease.
Its diagnosis can be challenging, as the disease must be differentiated from lymphoma and systemic lupus erythematosus. Kikuchi fujimoto disease is an inflammatory selflimited disorder of unknown etiology, characterized by systemic symptoms commonly associated with leukopenia. Kikuchi fujimoto disease kfd is a rare, but important cause of fever and cervical lymphadenopathy as it can mimic several other serious conditions. Kikuchifujimoto disease has been associated with sle and other connective tissue diseases, such as antiphospholipid syndrome, sjogrens syndrome, relapsing polychondritis and autoimmune hepatitis.
Kikuchi first described the disease in 1972 in japan. Here, we describe a case of kfd which initially presented with fever of unknown origin. While the exact cause of this condition is unknown, infectious and autoimmune causes have been suggested. A diagnosis of kikuchi fujimoto disease was rendered. Histopathology of the involved lymph nodes differentiates kikuchi disease from several more. Mar 22, 2014 the disease is characterised by a unilateral posterior cervical lymphadenopathy. Also called histiocytic necrotizing lymphadenitis, kikuchi fujimoto disease initially described in japan and other asian countries. The diagnosis of kikuchifujimoto disease is made predominantly on basis of morphologic evaluation. Less frequent symptoms include weight loss, nausea, vomiting. Histologically, the dermatological manifestations of kikuchi fujimoto disease have features in common with systemic lupus erythematosus.
Histiocytic necrotizing lymphadenitis, kikuchi disease, kikuchi fujimoto disease, kikuchi lymphadenitis, lymphadenopathy, kfd. Piriformis pyomyositisina patient withkikuchifujimoto. Kikuchi fujimoto disease kfd or histiocytic necrotizing lymphadenitis was first described in japan in 1972 1, 2. Kikuchi disease is a benign disease of the lymph nodes.
Pdf kikuchifujimoto disease kfd is a benign and selflimited disorder, characterized by regional cervical lymphadenopathy with tenderness, usually. It most commonly affects adults younger than 40 years of age and of asian descent. Pdf a case of kikuchifujimoto disease associated with. Kikuchifujimoto disease radiology reference article. This patient did not receive therapy at the time of presentation of kikuchi fujimoto disease. 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. A case report from the city of biratnagar, eastern zone of nepal. Kikuchi disease, also called histiocytic necrotizing lymphadenitis or kikuchi fujimoto disease, is an uncommon, idiopathic, generally selflimited cause of lymphadenitis.
Recognition of this condition is crucial, especially because it can easily be mistaken for tuberculosis, lymphoma, or even adenocarcinoma. Lymphadenopathy syndrome in systemic lupus erythematosus. Lymph nodes are present throughout the body as small oval structures that filter lymph fluid, fight infection and form white blood cells and blood plasma cells. Kikuchifujimoto disease presenting after consumption of. Kikuchifujimoto disease associated to the epsteinbarr. Symptoms are usually nonspecific fever, night sweats, etc. Is recurrent kikuchifujimoto disease a precursor to systemic. Oct 15, 2019 kikuchi fujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare, benign, and selflimited disease of unknown etiology that causes lymphadenopathy and has a characteristic. Kikuchi fujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis is an uncommon, idiopathic, generally selflimited cause of lymphadenitis. Kikuchi fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among japanese and other asiatic individuals. Less common symptoms include weight loss, nausea, vomiting, and sore throat. Kikuchis disease nord national organization for rare. Enigmatic kikuchifujimoto disease american journal of.
Prevalence of fkd is high among japanese and other asiatic individuals, but only isolated cases are reported in europe. Kikuchis disease is a rare nonmalignant disorder that affects the lymph nodes. Sep 14, 2016 kikuchi disease is a benign noncancerous condition of the lymph nodes. Review article kikuchifujimoto disease associated with sjogrens syndrome. It was first reported in japan in 1972, and since, this disease has been described worldwide, although most cases have been reported in asia. Kikuchi fujimoto disease, known as histiocytic necrotizing lymphadenitis, is a benign, selflimiting. It is also known as histiocytic necrotizing lymphadenitis, kikuchi necrotizing. Kikuchifujimoto disease kfd, also known as kikuchi s disease or necrotizing granulomatous lymphadenitis, is rare, has a benign course and is of unknown cause.
Kikuchifujimoto disease masquerading as a complication from. 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 fujimoto disease is a benign and usually selflimiting disease that mainly affects women kikuchi fujimoto disease kfd, also known as histiocytic necrotising lymphadenitis, is a rare clinical condition that can be difficult to diagnose due to its lack of specific signs, symptoms and serological markers. Kikuchifujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is a relatively rare condition characterized by subacute necrotizing regional lymphadenopathy. A rare association rinuchitra d 1, jagadeeswaran govindarajan, saravanan thangavelu, alamelu jayaraman 2 1 department of medicine and 2 department of pathology, psg hospitals, institute of medical science and research, coimbatore, india. First reported in japan, there has been an increase in its incidence globally. This disease was first described by both kikuchi and fujimoto in japan in early 1970s 3,4. Our report describes a 35yearold woman who presented with fever and cervical lymphadenopathy. Because of its nonspecific clinical features, the disease can be easily mistaken for other forms of lymphadenitis with a high rate of misdiagnosis. Kikuchi disease, is a rare pathology of the lymph nodes. The aetiology of kikuchi fujimoto disease remains controversial, but viral, autoimmune and physicochemical causes have been suggested as possibilities. Kikuchi disease or histiocytic necrotizing lymphadenitis is a rare lymph node disorder originally described in japan in 1972. My 30 year old son has been but there is so little information on it and the drs he has seen its the first time they have heard of it. Pdf kikuchi disease was described in 1972 in japan.
Kikuchi fujimoto disease associated to the epsteinbarr virus. Antinuclear antibodies, antiphospholipid antibodies, antidsdna, and rheumatoid factor are usually negative, and may help in differentiation from systemic lupus erythematosus. It is a rare cause of lymphadenopathy, commonly seen in individuals of asian descent and frequently associated with fever. Kikuchifujimoto disease is an uncommon and sometimes familial disorder that should be considered in the differential diagnosis of cervical lymphadenopathy. Kikuchi fujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a benign and selflimited disease that mainly affects young women. Kikuchi disease is a selflimiting illness which has symptoms which may overlap with hodgkins lymphoma leading to misdiagnosis in some patients.
The aetiology is poorly understood, but it appears to be an autoimmune disorder with a possible infectious trigger. A type of rare necrotizing lymphadenitis and its differential diagnosis 187 where a viral origin is assumed, are kawasaki disease and kikuchi fujimoto disease histiocytic necrotizing lymphadenitis. Although kikuchifujimoto disease is selflimiting and no. Oct 21, 2019 kikuchi fujimoto disease, known as histiocytic necrotizing lymphadenitis, is a benign, selflimiting and systemic disorder involving lymph nodes with unknown aetiology.
We report the case of a 27yearold argentinian female patient without any relevant past medical history to demonstrate the correlation between kikuchifujimoto disease and systemic lupus erythematosus. Kikuchifujimoto disease, or histiocytic necrotizing lymphadenitis, is a selflimited condition, characterized by benign lymphadenopathy with associated fevers and systemic symptoms. Kikuchi s disease seems to be more prevalent in japanese and asian individuals. Kikuchi s disease is a rare, benign and selflimiting disease involving lymph nodes. Kikuchi fujimoto s disease kfd, or histiocytic necrotizing lymphadenitis, is a benign and selflimiting disease of unknown aetiology. The cause of kikuchi fujimoto s disease is unknown.
Patients can present with nonspecific cutaneous lesions. Kikuchi fujimoto disease kfd, or histiocytic necrotizing lymphadenitis, is a rare, benign, and selflimited disease that causes lymphadenopathy and has a characteristic histological appearance. Kikuchifujimoto disease is selflimiting with acute to subacute course, evolving during several weeks. A higher prevalence of this disease is seen among the asian population. Kikuchi disease genetic and rare diseases information. Since kfd was first reported in 1972, the validity of this clinical entity has been controversial and its aetiology remains unknown.
Kikuchifujimoto disease kfd, also known as kikuchi disease, is a rare lymphohistiocytic disorder first described in 1972. Kikuchifujimoto disease blood american society of hematology. Kikuchi s disease or the kikuchi fujimoto disease kfd is a very rare, selflimiting, benign form of histiocytic necrotizing lymphadenitis, which is mostly seen in young females. Kikuchifujimoto disease is a selflimiting disease which frequently appears with fever and lymphadenopathy, thus creating the need for differential diagnosis of persistent febrile lymphadenopathy.
Patients present with localized lymphadenopathy, fever, and leukopenia in up to half of the cases. Kikuchifujimoto disease kfd is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, subacute necrotizing lymphadenitis, kikuchi lymphadenitis, and kikuchi fujimoto disease, is an uncommon cause of lymphadenopathy. Now he has a softball size lymph node in his abdomen that the drs think might be kikuchi disease. Due to its nonspecific symptoms and low incidence, kfd. Kikuchi fujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is a rare cause of prolonged cervi cal lymphadenopathy in both the pediatric and adult populations. Now he has a softball size lymph node in his abdomen that the drs think might be kikuchi disease again. The disease was described in 1972 by kikuchi 1 and independently by fujimoto et al 2. Pdf kikuchifujimoto disease in a young african american.
Lactate dehydrogenase and alanine aminotransferase concentrations can be raised. Typical presentation includes fever, leukopenia and cervical lymphadenopathy. The disease was initially described in 1972, in japan, in young females affected by fever and 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. Kikuchifujimoto disease and systemic lupus erythematosus imcrj. Lymph nodes involuted spontaneously on 6month followup without therapy. Kikuchi fujimoto disease histiocytic necrotizing lymphadenitis cervical adenopathy fever summary kikuchi fujmoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is a rare lymphadenitis of unknown origin, but with an excellent prognosis. Patients most commonly present with posterior cervical lymphadenopathy 60%90% of cases, frequently with concomitant involvement of axillary andor supraclavicular lymph nodes.
Kikuchifujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a rare and selflimited syndrome characterized by tender cervical. Kikuchi fujimoto disease kfd is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. Kikuchi fujimoto disease masquerading as a complication. Tuberculosis tb is a highly prevalent disease in peru. Kikuchi fujimoto disease kfd is typically a selflimited, benign illness which presents with fever and lymphadenopathy. The following cases have burn reported for clinical interest and for the rarity of the reports about this condition in ent literature. Kikuchi disease is a benign self limiting condition of unknown etiology, although infectious and autoimmune causes have been suggested 46. In february, 20, a 30yearold ugandanborn woman, working in.
Kikuchi fujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is an extremely rare, selflimiting disorder which typically presents with fever and painful, unilateral cervical lymphadenopathy in previously healthy individuals. Kikuchi fujimoto disease is a hyperimmune reaction of t cells which triggers cellular degeneration of necrosis. This is a benign and selflimited disease, frequently associated with mild fever, and occasionally with other systemic symptoms. Less frequent symptoms include weight loss, nausea, vomiting, sore throat. Fujimoto and colleagues described kikuchi s disease in the same year. A diagnosis of kikuchi fujimoto disease was established via lymph node biopsy after other differentials were excluded.
Kikuchi fujimoto disease is an idiopathic, extremely rare, more or less worldwide, and often underdiagnosed condition. Kikuchi fujimoto disease also known as histiocytic necrotizing lymphadenitis is an idiopathic benign self limiting condition with a favourable outcome, usually affecting young women. Kikuchifujimoto disease, known as histiocytic necrotizing lymphadenitis, is a benign, selflimiting and systemic disorder involving lymph nodes with unknown aetiology. 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. It is rare in caucasians, normally occurring in those of asian descent. Mar 15, 2006 kikuchifujimoto disease kfd was first described in japan in 1972. As in many benign and malignant lymph node disorders, lowpower pattern recognition is critical in this disease. Immunohistochemical staining is sometimes useful, especially in cases with limited tissue ie, core biopsies or small. Kfd has a worldwide distribution, and asiatic people have a higher prevalence. This disease is misdiagnosed as malignant lymphoma in up to onethird of cases and is as sociated with the development of systemic lupus erythematosus sle. Pathogenesis, diagnosis, and management of kikuchi. Pathogenesis, diagnosis, and management of kikuchifujimoto. May 23, 2006 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.
Fujimoto disease kfd is an important cause of lymphadenitis in children. The resulting nuclear dust or karyorrhectic debris is phagocytosed and degraded by histiocytes which are plasmacytoid mononuclears that stain with macrophage reagents. Histiocytic necrotizing lymphadenitis, kikuchi disease, kikuchifujimoto disease, kikuchi lymphadenitis, lymphadenopathy, kfd kfd is a rare lymphohistiocytic disorder due to cervical inflammatory lymphadenitis or axillary region and even other location seldom with an unknown etiopathogenesis, that is. Kikuchifujimoto disease kfd is a rare, selflimiting disorder that typically affects the cervical lymph nodes.
The main sign of kikuchi disease is usually swollen lymph nodes in the neck, which tend to develop suddenly. Is recurrent kikuchifujimoto disease a precursor to. Kikuchi fujimoto disease kfd is a rare form of painful lymphadenopathy, usually cervical, which is more common in southeast asia and rarely reported from africa. Kfd tends to affect a young population under 30 years of age. Kikuchi fujimoto disease usually presents as a febrile illness with unilateral enlarged cervical lymphadenopathy of less than 2 cm. He had it in his neck in february 2017 it was removed and he got better.
Kikuchifujimoto disease histiocytic necrotizing lymphadenitis cervical adenopathy fever summary kikuchifujmoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is a rare lymphadenitis of unknown origin, but with an excellent prognosis. Review article kikuchifujimoto disease associated with. The clinical, histopathological and immunohistochemical features appear to point to a viral etiology, a hypothesis that. Awareness of this disorder helps prevent misdiagnosis and inappropriate treatment. Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, is a selflimiting benign lymphadenopathy of unknown origin that predominantly affects young adults. The disease frequently mimics tuberculous lymphadenitis, malign lymphoma, and many other benign and malignant conditions. 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%. The main symptoms include swollen lymph nodes in the neck, mild fever, and night sweats. Jan 11, 2011 kikuchifujimoto disease has been found in association with various viruses, bacteria and parasites, but as of yet no clear causal relation has been shown. Kikuchi fujimoto disease fkd, also called histiocytic necrotizing lymphadenitis, is a rare, idiopathic and generally selflimiting cause of lymphadenitis. The primary aim of this study was to investigate the clinical characteristics of children with kfd and to assess the recurrence of this disease. Kikuchi disease is characterized histopathologically by. Kikuchifujimoto disease as a differential diagnosis for lymph. 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.
Dec 09, 2017 my 30 year old son has been but there is so little information on it and the drs he has seen its the first time they have heard of it. Kikuchi fujimoto disease kfd, also known as histiocytic necrotizing lymphadenitis, is a rare, benign, selflimiting disease characterized by cervical lymphadenopathy and fever. 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. Kfd can occur in association with systemic lupus erythematosus. Kikuchi disease has been found to be associated with several autoimmune diseases, especially systemic lupus erythematous sle or lupus.
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