Clinical features and recurrence predictors of histiocytic necrotizing lymphadenitis in Chinese children.

OBJECTIVES: To characterize the clinical features and to identify the predictors of recurrence of histiocytic necrotizing lymphadenitis (HNL) in Chinese children. STUDY DESIGN: This study retrospectively analyzed the clinical characteristics, laboratory and pathological findings, and recurrence status of children diagnosed with HNL at a single center in China from January 2018 to May 2023. Logistic regression analysis was employed to identify predictors of HNL recurrence. RESULTS: 181 Chinese children with histopathologically confirmed HNL were enrolled (121 males and 60 females). The mean age was 9.3 ± 2.9 years. The most prominent clinical features were fever (98.9%) and cervical lymphadenopathy (98.3%). Aseptic meningitis was the most frequent complication (38.5%), while hemophagocytic lymphohistiocytosis and autoimmune disease were rare (1.7% and 1.2%, respectively). Recurrence occurred in 12.7% of patients. Erythrocyte sedimentation rate (> 30 mm/h) was the significant predictors of HNL recurrence, with odds ratios of 6.107, respectively. CONCLUSION: Our study demonstrates that fever and cervical lymphadenopathy are the most frequent clinical manifestations of HNL in Chinese children, which often coexist with aseptic meningitis. HNL patients with risk factors require follow-up for recurrence.

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.

Evaluation of macrophage activation syndrome in hospitalised patients with Kikuchi-Fujimoto disease based on the 2016 EULAR/ACR/PRINTO classification criteria.

BACKGROUND: To evaluate the impact of macrophage activation syndrome (MAS) on clinical features in patients with Kikuchi-Fujimoto disease (KFD) and to compare the features of MAS in KFD with those of adult-onset Still's disease (AOSD) and systemic lupus erythematosus (SLE). METHODS: The medical records of febrile patients hospitalised with KFD between November 2005 and April 2017 were reviewed. Patients fulfilling the 2016 classification criteria for MAS were classified as having MAS. Clinical and laboratory features of patients with KFD with and without MAS were evaluated. Poor hospitalisation outcomes were defined as intensive care unit admission or in-hospital mortality. The treatment outcomes of MAS in KFD, AOSD, and SLE were also compared. RESULTS: Among 78 patients hospitalised with KFD, 24 (30.8%) patients had MAS during admission. Patients with KFD and MAS more frequently required glucocorticoid treatment (66.7% vs 40.7%, p = 0.036) and had longer hospital stays than patients with KFD without MAS (12.5 vs 8.5 days, p<0.001). In addition, patients with MAS had worse hospitalisation outcomes than patients without MAS (29.2% vs. 0.0%, p<0.001). Among patients with MAS in KFD, AOSD, and SLE, the number of patients requiring glucocorticoid treatment after 3 months was significantly lower among patients with MAS and KFD (KFD 33.3%, AOSD 88.9%, SLE 100%, p<0.001). CONCLUSIONS: The presence of MAS in KFD was associated with adverse clinical outcomes including higher steroid usage and worse hospitalisation outcomes. However, compared to those with AOSD and SLE, patients with MAS and KFD were less likely to require long-term glucocorticoid treatment.

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.

Salmonella Enteritidis ST183: emerging and endemic biotypes affecting western European hedgehogs (Erinaceus europaeus) and people in Great Britain.

The impacts of hedgehog (Erinaceus europaeus) Salmonella infection on public health and on animal welfare and conservation are unknown. We isolated Salmonella Enteritidis multi-locus sequence-type (ST)183 from 46/170 (27%) hedgehog carcasses (27 S. Enteritidis phage type (PT)11, 18 of a novel PT66 biotype and one with co-infection of these PTs) and from 6/208 (3%) hedgehog faecal samples (4 PT11, 2 PT66) from across Great Britain, 2012-2015. Whole genome phylogenetic analysis of the hedgehog isolates and ST183 from people in England and Wales found that PT11 and PT66 form two divergent clades. Hedgehog and human isolates were interspersed throughout the phylogeny indicating that infections in both species originate from a common population. PT11 was recovered from hedgehogs across England and Scotland, consistent with endemic infection. PT66 was isolated from Scotland only, possibly indicating a recent emergence event. People infected with ST183 were four times more likely to be aged 0-4 years than people infected by the more common ST11 S. Enteritidis. Evidence for human ST183 infection being non-foodborne included stronger correlation between geographic and genetic distance, and significantly increased likelihood of infection in rural areas, than for ST11. These results are consistent with hedgehogs acting as a source of zoonotic infection.

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.

Clinical Characteristics of Severe Histiocytic Necrotizing Lymphadenitis (Kikuchi-Fujimoto Disease) in Children.

OBJECTIVES: To analyze the clinical characteristics of children with Kikuchi-Fujimoto disease focusing on cases with prolonged fever. STUDY DESIGN: This was a retrospective study of children diagnosed with Kikuchi-Fujimoto disease from March 2003 to February 2015 in South Korea. Electronic medical records were searched for clinical and laboratory manifestations. RESULTS: Among 86 histopathologically confirmed cases, the mean age was 13.2 (SD ± 3.1) years, and male to female ratio was 1:1.32. Cervical lymph node enlargement, found in 85 of the patients (99%), was predominantly unilateral in 64 (75%), and involved the cervical lymph node level V in 67 (81%). Fever was present in 76% of the cases, with a median duration of 9 days (IQR 0.25-17.0). Multivariate analysis revealed that a high fever peak ≥ 39.0°C (P = .010) and presentation with ≥ 2 systemic symptoms other than fever (P = .027) were factors that were significantly associated with longer fever duration. As the size of the largest lymph node's short diameter increased, the fever duration increased (P = .015). Leukopenia (P = .022) also had a significant association with a longer fever duration. Patients with sonographic findings of conglomerated enlarged lymph nodes had a longer median duration of fever compared with those with separate enlarged lymph nodes (11 vs 4.5 days, P = .019). CONCLUSIONS: Patients with high fever, more systemic symptoms, leukopenia, and larger lymph nodes with a conglomerated distribution may benefit from early recognition and selective consideration of corticosteroid therapy.

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.

Kikuchi-Fujimoto disease: retrospective study of 91 cases and review of the literature.

Kikuchi-Fujimoto disease (KFD) is a rare cause of lymphadenopathy, most often cervical. It has been mainly described in Asia. There are few data available on this disease in Europe. We conducted this retrospective, observational, multicenter study to describe KFD in France and to determine the characteristics of severe forms of the disease and forms associated with systemic lupus erythematosus (SLE). We included 91 cases of KFD, diagnosed between January 1989 and January 2011 in 13 French hospital centers (median age, 30 ±â€Š10.4 yr; 77% female). The ethnic origins of the patients were European (33%), Afro-Caribbean (32%), North African (15.4%), and Asian (13%). Eighteen patients had a history of systemic disease, including 11 with SLE. Lymph node involvement was cervical (90%), often in the context of polyadenopathy (52%), and it was associated with hepatomegaly and splenomegaly in 14.8% of cases. Deeper sites of involvement were noted in 18% of cases. Constitutional signs consisted mainly of fever (67%), asthenia (74.4%), and weight loss (51.2%). Other manifestations included skin rash (32.9%), arthromyalgia (34.1%), 2 cases of aseptic meningitis, and 3 cases of hemophagocytic lymphohistiocytosis. Biological signs included lymphocytopenia (63.8%) and increase of acute phase reactants (56.4%). Antinuclear antibodies (ANAs) and anti-DNA antibodies were present in 45.2% and 18% of the patients sampled, respectively. Concomitant viral infection was detected in 8 patients (8.8%). Systemic corticosteroids were prescribed in 32% of cases, hydroxychloroquine in 17.6%, and intravenous immunoglobulin in 3 patients. The disease course was always favorable. Recurrence was observed in 21% of cases. In the 33 patients with ANA at diagnosis, SLE was known in 11 patients, diagnosed concomitantly in 10 cases and in the year following diagnosis in 2 cases; 6 patients did not have SLE, and 4 patients were lost to follow-up (median follow-up, 19 mo; range, 3-39 mo). The presence of weight loss, arthralgia, skin lesions, and ANA was associated with the development of SLE (p < 0.05). Male sex and lymphopenia were associated with severe forms of KFD (p < 0.05). KFD can occur in all populations, irrespective of ethnic origin. Deep forms are common. An association with SLE should be investigated. A prospective study is required to determine the risk factors for the development of SLE.

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.

Characteristics of Kikuchi-Fujimoto disease in children compared with adults.

Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting disease characterized by cervical lymphadenopathy. Although it was primarily thought to be a disease of young adults, it has been increasingly recognized in children. To define the characteristics of KFD in children, we reviewed the medical records of patients younger than 18 years of age who were diagnosed with KFD from 2001 to 2012 at Korea University Medical Center, as well as worldwide published reports of KFD. A total of 140 pediatric patients and 733 patients of all ages was analyzed. Compared to the female predominance found in adults (2:1), young boys were more commonly affected than young girls (1.4:1). Cervical lymphadenopathy was the most common clinical finding in children, as it was in adults. Lymphadenopathy was more likely to be tender (69 vs. 44 %, p < 0.001) but less generalized (1 vs. 8 %, p < 0.05) in children compared to adults. Fever (82 vs. 35 %, p < 0.001) and rash (10 vs. 4 %, p < 0.05) were observed in children more commonly than in adults. Leukopenia was observed in 50 and 38 % of children and adults, respectively. Rates of recurrence and association with autoimmune diseases in children were comparable to those of adults. Cervical lymphadenopathy was the most common clinical manifestation of KFD in all ages. While fever and rash were more common in children with KFD compared to adults, generalized lymphadenopathy was rarer.

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.

Histiocytic necrotizing lymphadenitis in the context of systemic lupus erythematosus (SLE): Is histiocytic necrotizing lymphadenitis in SLE associated with skin lesions?

Histiocytic necrotizing lymphadenitis (HNL), or Kikuchi's disease, is a benign and self-limiting lymphadenopathy that typically affects young Asian females. It presents with lymphadenopathy, usually cervical, accompanied by fever, chills and leukopenia. Although the association between systemic lupus erythematosus (SLE) and HNL is rare, the number of reports of HNL in SLE patients is increasing. We present nine cases of HNL in patients with SLE. Among the seven patients with diverse skin manifestations, three had skin manifestations that were histologically compatible with SLE. A review of previous reports in the literature showed that cutaneous involvement was commonly found in HNL in association with SLE. In the patients who had simultaneous onset of both diseases, lupus flare-ups were commonly observed. We suggest that HNL in SLE patients is associated with cutaneous manifestations. This report contributes to our understanding of the relationship between these diseases.