Kikuchi-Fujimoto disease in the Eastern Mediterranean zone.

Kikuchi-Fujimoto disease (KFD) is a rare benign and self-limiting syndrome. We aim to review cases of KFD at our institution as a rare illness in the Arab ethnic descent and to analyse reports from most countries in the East Mediterranean zone. This is a retrospective study in which the histopathology database was searched for the diagnosis of KFD. A full review of KFD patients' medical records was done. Data regarding demographic features, clinical presentation, laboratory findings, comorbidities, and management protocols were obtained. Published KFD cases from east Mediterranean countries were discussed and compared to other parts of the world. Out of 1968 lymph node biopsies studied, 11 (0.6%) cases of KFD were identified. The mean age of patients with KFD was 32 years (4-59). 73% (8/11) were females. The disease was self-limiting in 5 patients (45%); corticosteroid therapy was needed in 4 patients (34%). One patient was treated with methotrexate and one with antibiotics. One patient died as a consequence of lymphoma. Jordanians and Mediterranean populations, especially those of Arab ethnic background, seem to have low rates of KFD. The genetic susceptibility theory may help to explain the significantly higher disease prevalence among East Asians. Early diagnosis of KFD-although challenging-is essential to reduce the morbidity related to this illness.

Kikuchi-Fujimoto disease in children.

AIM: Kikuchi-Fujimoto disease (KFD) is an important cause of lymphadenitis in children. The primary aim of this study was to investigate the clinical characteristics of children with KFD and to assess the recurrence of this disease. METHODS: This is a retrospective study of patients younger than 18 years old, who were diagnosed with KFD from January 2000 to September 2017 at KK Women's and Children's Hospital. Records of children with a histological diagnosis of KFD from a lymph node biopsy were obtained from the Department of Pathology. Case notes and electronic medical records of the patients were reviewed. Data collected included patient characteristics, symptoms, clinical and laboratory findings, treatment and follow-up. RESULTS: A total of 98 patients were identified. There were 52 boys and 46 girls with a median age of 11.2 years old. Recurrence occurred in 12 (12.2%) patients. One patient developed systemic lupus erythematosus 10 years after diagnosis of KFD. Recurrent cases were more likely to be managed as an inpatient and have fever at presentation of their first episode of KFD. CONCLUSION: In our study, KFD in children had a higher prevalence among boys, and had a recurrence rate of 12.2%, with 1% of patients developing systemic lupus erythematosus. We recommend that patients be followed up for recurrence and advised to monitor for symptoms of recurrence.

Rare diseases that mimic Systemic Lupus Erythematosus (Lupus mimickers).

Several conditions have clinical and laboratory features that can mimic those present in Systemic Lupus Erythematosus (SLE). Some of these "SLE mimickers" are very common, such as rosacea which can be mistaken for the butterfly rash, while others such as Kikuchi disease, type-1 interferonopathies, Castleman's disease, prolidase deficiency, angioimmunoblastic T-cell lymphoma, Evans' syndrome in the context of primary immune deficiencies and the autoimmune lymphoproliferative syndrome are exceptionally uncommon. A proper diagnosis of SLE must therefore be based upon a complete medical history as well as on the adequate constellation of clinical or laboratory findings. While there is no single test that determines whether a patient has lupus or not, the search for auto-antibodies towards nuclear antigens is a key step in the diagnosis strategy, keeping in mind that ANAs are not specific for SLE. In case of persistent doubt, patients should be referred to reference centers with experience in the management of the disease.

The incidence and clinical characteristics by gender differences in patients with Kikuchi-Fujimoto disease.

Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting disorder that typically affects the cervical lymph nodes (LNs). Although initially described in young women, KFD also occurs in men. There are no reports on the clinical manifestations and characteristics of male KFD patients. Therefore, this study was conducted to assess the incidence of KFD among males, as well as the most frequent clinical characteristics of these patients. A retrospective, cross-sectional study was performed at a tertiary hospital of patients pathologically confirmed as having KFD from LN biopsy specimens. Clinical and laboratory data, and treatment outcomes of the enrolled patients, were analyzed by gender. A total of 254 patients diagnosed with KFD were enrolled. There were 189 females and 65 males (2.9:1). The mean age was 32.6 ±â€Š11.3 years. Compared to the female patients, the males had more frequent manifestations of fever (48% vs 67%, P = 0.008), headache (9% vs 20%, P = 0.013), bilateral lymphadenopathy (31% vs 46%, P = 0.029), thrombocytopenia (14% vs 29%, P = 0.014), elevated C-reactive protein (CRP) (35% vs 78.4%, P < 0.001), elevated liver enzymes (15% vs 41%, P < 0.001), and elevated lactate dehydrogenase (LDH) (61% vs 80%, P = 0.021). Male patients had fewer autoimmune features (9% vs 2%, P = 0.043) and fewer positive antinuclear antibodies (32% vs 10%, P = 0.006). In this study, 25.6% of the enrolled patients were male, with a 2.9:1 female-to-male sex ratio. Male patients showed a distinctive profile characterized by a higher frequency of fever, headache, bilateral lymphadenopathy, and thrombocytopenia, as well as elevated liver enzymes, CRP, and LDH.

Epidemiology and Characteristics of Kikuchi-Fujimoto Disease in the African-Descent Population of Martinique, French West Indies.

OBJECTIVE: To provide an epidemiologic description of Kikuchi-Fujimoto disease (KFD), and to describe its relationship with systemic lupus erythematosus (SLE) in a population of sub-Saharan origin. METHODS: Patients were retrospectively included on the basis of lymph node histology compatible with KFD reported in Martinique from 1991 until 2013. In order to describe the characteristics of the disease in a larger cohort, we subsequently included more patients of Afro-Caribbean origin from Guadeloupe and French Guiana. RESULTS: In Martinique, mean annual incidence between 1991 and 2013 was 2.78 cases for 1 million inhabitants (95% confidence interval 1.73-3.93). A total of 36 Afro-Caribbean patients from the 3 French American regions were included. Mean age was 30.5 years (range 5-59 years) and the female:male ratio was 3:1. The main characteristics were cervical adenopathies (88.8%), fever (83.3%), asthenia (73.0%), weight loss (64.4%), and recurrence in 33.3%. KFD was associated with lupus (n = 9 for SLE, n = 2 for cutaneous lupus) in 36.6% (11 of 30). CONCLUSION: We report the first epidemiologic description of KFD in a population of sub-Saharan origin. According to our data, this disease is present in the black African diaspora and is strongly associated with autoimmune diseases, particularly lupus.

Kikuchi-Fujimoto disease: a clinicopathologic update.

Kikuchi-Fujmoto disease (KFD), also known as "histiocytic necrotizing lymphadenitis", is a rare lymphadenitis of unknown origin, but with an excellent prognosis. It is more common in Asia, but isolated cases are also reported in America, Africa and Europe. The disease can have an acute or subacute course, usually develops in 1 to 3 weeks, with spontaneous resolution in 1-4 months. The main clinical sign is cervical lymphadenopathy, especially in the posterior cervical triangle with bulky and painful lymph nodes, usually affecting only one side; rare cases of generalized lymphadenopathy can be seen. This common clinical presentation can also be accompanied by nausea, vomiting, weight loss, weakness, headache and arthralgia. An extranodal extension of the disease, including involvement of skin, eye, and bone marrow localizations, has been rarely described. Most patients have leukopenia or neutropenia with a relative leukocytosis. At an ultrasound exploration of the affected lymph nodes, a hypoechoic aspect can be seen, with an external, thick and irregular hyperechoic ring. As there are no specific tests for KFD, the final diagnosis is histologically-based from lymph node excisional biopsy. Histological examination shows paracortical foci of coagulative necrosis containing karyorrhectic debris, which are surrounded by numerous CD68+/myeloperoxidase (MPO)+ histiocytes, CD68+/CD123+ plasmacytoid dendritic cells, and a minority of small- to large-sized CD8+lymphocytes and immunoblasts. Differential diagnosis mainly includes systemic lupus erithematous (SLE)-related lymphadenopathy and large cell lymphoma. The histological absence of neutrophils, plasmacells, as well as hematoxylin bodies, is a feature which argues against the diagnosis of SLE. In addition, the absence of auto-antibodies and anti-nuclear antibodies is useful in ruling out an autoimmune disorder. Early diagnosis of KFD is crucial to prevent the patients undergo extensive investigations related to suspected malignant lymphomas or other diseases.

An unusual presentation of Kikuchi-Fujimoto disease.

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, self-limited disease of unknown etiology. This condition is most commonly encountered in Asian and Caucasian females of childbearing age who usually present with cervical lymphadenopathy and fever. Though rarely, KFD has been described in younger African-American females as well. It often mimics more serious conditions such as lymphoma, metastatic solid malignancy, HIV infection, tuberculosis, sarcoidosis, or systemic lupus erythematosus. Although its etiopathogenesis has not been fully elucidated, literature suggests viral or possibly autoimmune components to play a role. We describe a 34-year-old African-American female who presented with constitutional symptoms and polyadenopathy on clinical examination and imaging, of which the portacaval and portahepatis lymph nodes were most prominent. An extensive workup was otherwise unremarkable, and biopsy showed histiocytic necrotizing lymphadenitis. Initially, her clinical condition improved spontaneously, and she required only a short course of oral steroids. Three months later, she relapsed with bilateral cervical adenopathy and constitutional symptoms and was successfully managed again with steroids. Our case is unique with respect to (a) portahepatis and portacaval node enlargement as the dominant adenopathy and (b) her underlying conditions of fibromyalgia and chronic fatigue syndrome.

Comparison of clinical features and EBV expression in histiocytic necrotizing lymphadenitis of children and adults.

OBJECTIVE: Histiocytic necrotizing lymphadenitis (HNL) is a self-limiting disease characterized by cervical lymphadenopathy and fever. Epstein-Barr virus (EBV) has been implicated as a cause of HNL in some reports but not in others. The aim of this study was to compare the clinical features and laboratory findings in children and adults with HNL and to evaluate the relationship between EBV infection and HNL. METHODS: The records of patients diagnosed with biopsy-confirmed HNL at Gyeongsang National University Hospital from May 2009 to December 2012 were retrospectively reviewed. Clinical features, laboratory data (blood count, biochemistry, and serology), and computed tomography findings were collected. Immunohistochemistry for CD4, CD8, CD68, and myeloperoxidase (MPO), and in situ hybridization for EBV RNA were performed on archived lymph node biopsies from pediatric and adult patients. RESULTS: A total of 16 children and 31 adults were diagnosed with HNL. Fever was present in 87.5% of children and 25.8% of adults (P<0.0001). Fever duration was longer for children (mean, 5.06 days) than for adults (mean, 0.63 days; P=0.008). Necrosis of the involved lymph nodes was observed in eight (50%) children and six (19.4%) adults (P=0.045). HNL recurred in five (31.35%) children and one (3.2%) adult (P=0.015). All had detectable CD4, CD8, CD68, and MPO immunoreactivity. EBV reactivity was higher in children (26.7%) than in adults (6.7%) without statistical support. CONCLUSIONS: There is more variability in the clinical presentation and laboratory findings in children with HNL than in adults. In particular, the degree and duration of fever was more severe in children than in adults, and necrosis of lymph nodes was more frequent in children than in adults. There was no definitive correlation between EBV reactivity and NHL. Further study of the relationship between EBV and HNL is needed with a larger number of patients.

Kikuchi-Fujimoto disease: the first reported case in Croatia.

Kikuchi-Fujimoto disease (KFD) is an extremely rare disease known to have a worldwide distribution with higher prevalence among Japanese and other Asiatic individuals. KFD presents as benign and self-limited disorder, characterized by regional cervical lymphadenopathy with tenderness, usually accompanied with mild to high fever and night sweats. Less frequent symptoms include weight loss, nausea, vomiting and sore throat. Final diagnosis can only be determined on the basis of typical morphological changes in the lymph node, therefore lymph node biopsy is crucial for proving the diagnosis. Here we present a 16-year-old, native Croatian, Caucasian girl with KFD, as a first case of KFD reported in Croatia. We suggest that this disease should be considered as a possible cause of fever of the unknown origin followed by lymphadenopathy.

Kikuchi-Fujimoto's disease: a case series from Sri Lanka.

INTRODUCTION: Kikuchi's disease is a rare, benign, self-limiting disease, mainly involving the lymph nodes of young people. The etiology is unknown. Clinical symptoms and basic investigations may mimic lymphomas and chronic granulomatous conditions like tuberculosis. Lymph node biopsy shows characteristic diagnostic features. Even though described internationally, the local disease pattern or incidence has not been well studied. METHODS: We studied all patients who were diagnosed with Kikuchi's disease at Teaching Hospital, Peradeniya from January 2011 to April 2012. RESULTS: A total of 9 cases showed histopathological features of Kikuchi's disease. All patients were females, in the age group of 12-30 years having fever and lymphadenopathy. They carried a provisional diagnosis of lymphoma, tuberculosis or reactive lymphadenitis. CONCLUSIONS: Necrotising lymphadenitis has a predilection for cervical lymph nodes of females and is usually accompanied by fever. Clinical features can resemble tuberculous lymphadenitis or malignant lymphoma. Excision biopsy of the involved node is mandatory for the diagnosis.

Recurrent histiocytic necrotizing lymphadenitis with a long latency in a patient with autoimmunity: a case report and review of literature.

Kikuchi-Fujimoto disease (KFD), a histiocytic necrotizing lymphadenitis (HNL), characteristically presents as cervical lymphadenopathy in young Asian women. Most resolve spontaneously with rare recurrences described. We report a patient with biopsy-proven recurrence of KFD-like HNL after almost 8 years and analyze 65 additional published cases with recurrences. While those with recurrences similarly affect young (average age = 27 years), Asian (80%) women (76%), 73% had multiple sites of involvement and 32% of those tested had underlying autoimmune conditions. Our case is unusual with respect to the following: (a) Age: 50 years, the oldest among the reported patients with recurrences. (b) Race: African descent, with only 3 others reported with recurrent HNL. Of these 4 cases, 2 had underlying autoimmunity. (c) Underlying condition: Her clinical and laboratory features were best felt to represent Sjögren's syndrome (SjS). Only 2 other cases of SjS-associated HNL have been reported; in 2 recently reported cases SjS developed subsequently.

Clinicopathological review of immunohistochemically defined Kikuchi-Fujimoto disease-including some interesting cases.

Kikuchi-Fujimoto Disease (KFD) is a benign, self-limited disease characterized by tender regional lymphadenopathy with fever. KFD remains a poorly defined disease, and no clear diagnostic criteria are available. Here, we assess the clinical, laboratory, and histopathologic findings of KFD cases and report two unusual cases. Forty KFD patients that underwent lymph node (LN) biopsy and diagnosed by immunohistochemical staining, from January 2003 to November 2010, were enrolled in this retrospective study. The patients had a mean age of 29.3 years, and 29 (72.5 %) were women. Affected LNs were mainly located unilaterally in the cervical area. Mean LN size was 15.3 mm. Twenty-eight (70 %) patients had LN tenderness, and 25 (62.5 %) patients had fever. Leukopenia was observed in 18 of 35 evaluable patients. C-reactive protein and erythrocyte sedimentation rate were elevated in most patients. Anti-nuclear antibody was positive in four of 19 evaluable patients, but all had been diagnosed with concurrent systemic lupus erythematosus. Histologically, the 40 cases were classified into three types, that is, as proliferative (37.5 %), necrotizing (55.0 %), or xanthomatous (7.5 %). Interesting cases: Case 1 was a 35-year-old female with KFD and uveitis, retinal vasculitis, and superior sagittal sinus thrombosis. Case 2 was a 47-year-old male with KFD and bone marrow involvement and presented with severe bicytopenia. Although KFD is an uncommon self-limited benign disorder, it must be included in the differential diagnosis of lymphadenopathy with fever and cytopenia. It is important that the clinical features of KFD be understood to reach a correct diagnosis.

[Kikuchi-Fujimoto disease]. / La maladie de Kikuchi-Fujimoto.

Kikuchi-Fujimoto disease is a necrotizing lymphadenitis, involving young patients, predominantly females. Lymphadenopathy is usually localized, particularly in the cervical area, mostly unilateral and tender. Fever is present in one third of cases. Associated skin lesions, arthralgia, myalgia, splenomegaly or hepatomegaly are rare. Laboratory evaluation shows a slight increase of erythrocyte sedimentation rate and leukopenia. Kikuchi-Fujimoto has been reported in association with other diseases, including systemic lupus, Still's disease, hemophagocytosis, pregnancy, other autoimmune diseases, and cancer. A viral or bacterial origin has been suspected but not confirmed. Lymph node biopsy allows the diagnosis and shows necrotizing lymphadenitis with acidophil necrosis, CD68+ histiocyte infiltrate, presence of plasmacytoid monocytes, multiple apoptotic cells (CD8+ T cell) with nuclear dust, immunoblastic reaction and the absence of neutrophils or eosinophils. The disease course is usually spontaneously favourable in few weeks or months, requiring corticosteroids only occasionally.

Childhood Kikuchi-Fujimoto disease.

To investigate the clinical features of Kikuchi-Fujimoto disease (KFD) and the relationship between viral infection and this disease in children will be better as Kikuchi-Fujimoto disease (KFD) is a lymphadenopathy. The aim of study is to investigate the clinical features of KFD and the relationship. The age, gender, clinical features and aetiopathogenesis of 36 Chinese children with FKD were reviewed, and the viral antigens were detected. Mean age was 10.1 +/- 2.8 yr with a male to female ratio of 1.8:1. Fever and lymphadenopathy were the most common complaints, noted in 23 and all cases respectively. Skin rash and hepatosplenomegaly were also noted. Leukopenia, anemia, thrombocytopenia and raised ESR were noted in 21, 6, 4 and 31 cases respectively. Epstein-Barr virus (EBV) IgM and IgG was positive in 1 and 24 of 29 cases respectively. Antigens of EBV and herpes simplex virus 2 (HSV 2) were found in the biopsy tissue section from 2 and 1 case respectively. Autoantibodies were noted in 3 of 15 cases. Steroid hormones were administrated for 19 cases with good efficacy. These results imply that children with lymphadenopathy and/or fever may have KFD and thus excisional biopsy of lymph nodes should be performed earlier on. A hyperimmune reaction of immune cells to EBV and HSV2 may play a role in the pathology of KFD.

Enfermedad de Kikuchi - Fujimoto: reporte de un caso / Kikuchi - Fuijimoto disease: report a case

La enfermedad de Kikuchi o Kikuchi Fujimoto o linfadenitis histiocítica necrotizante, es una rara y habitualmente benigna enfermedad que se presenta con fiebre y adenopatías cervicales usualmente en mujeres jóvenes. La etiología es desconocida, aunque hay evidencias de su origen viral y la semejanza del grupo poblacional afectado con el lupus eritematoso sistémico ha alentado, en base a estudios celulares, la sospecha de que el síndrome de Kikuchi refleja una condición Lupus - símil auto limitada y causada por linfocitos transformados por una infección viral. Inicialmente descripta en Japón, la enfermedad de Kikuchi Fujimoto se ha reportado en diferentes grupos étnicos y raciales, aunque ha sido predominantemente descripto en Asia. La enfermedad puede ser sospechada por la clínica pero se necesita de la anatomía patológica para su confirmación.

Kikuchi-Fujimoto lymphadenitis.

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 self-limited clinical course. 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, or ethnic background from a variety of geographic locations, including the United States. In addition, cases showing extranodal involvement have been described. The spectrum of clinical and histologic features associated with this disorder suggest that KFD more likely represents a common pattern of response to a variety of etiologic factors rather than a single clinicopathologic entity. In this review, the characteristic clinical, laboratory, histologic, and immunophenotypic features of this disorder are described, with emphasis on differential diagnosis with other types of necrotizing lymphadenitis and with malignant lymphoma. Unusual clinical presentations and features of disease when present at extranodal sites are also reviewed.

Histiocytic necrotizing lymphadenitis (Kikuchi's disease): clinicopathologic characteristics of 23 cases and literature review.

Kikuchi's disease or histiocytic necrotizing lymphadenitis is a self limiting disorder which usually affects young women and manifests clinically by cervical lymphadenopathy with or without fever. Though the disease has been described since 1972, many clinicians and pathologists are unaware of its existence. We therefore reviewed 23 cases of Kikuchi's disease in Songklanagarind Hospital from 1987 to 1996. Clinical data, histopathology including immunostaining were presented together with brief literature review. We found 18 women and 5 men with the age range 9-57 years. The sites of nodal enlargement were cervical in 17, axillary in 3 and unknown in 3 cases. Typical histologic features namely patchy paracortical lymphohistiocytic aggregates with variable karyorrhexis and absence of granulocytic infiltration were seen. Immunostaining confirmed that the principle cells were histiocytes and T-lymphocytes. Importance in the recognition of the entity was emphasized for it may be mistaken for other infective lymphadenitis or lymphoma.