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PubMed1990

Kikuchi's histiocytic necrotizing lymphadenitis.

Histiocytic necrotizing lymphadenitis (HNL) or Kikuchi's Disease is a peculiar condition most commonly affecting cervical lymph nodes in young women. Histology and immunohistochemistry demonstrate a florid T-cell and histiocytic reaction associated with necrotic areas which must be carefully distinguished from malignant lymphoma. Usually, the disease resolves spontaneously within a few weeks or months. The etiology is unknown and the physiopathology discussed.

Diagnosis, Differential↗
PubMed2003

[Kikuchi's histiocytic necrotizing lymphadenitis].

Necrotizing histiocytic lymphadenitis (NHL) or Kikuchi-Fujimoto disease is an infrequent seen clinicopathologic entity that affects most frequently young women and individuals of eastern races, and that is characterized by fever and adenopathies (basically cervical) and systemic disease even though occasional patients present extranodal disease. We present two young women with NHL and cutaneous affectation, and with lymphocytic meningitis in one of the patients. We discuss the etiology of NHL, the relation of NHL with collagen-vascular diseases (basically systemic erythematous lupus) the problem of the pathologic diagnosis, and the therapeutic possibilities in these patients.

Anti-Bacterial Agents↗
PubMed1999

[Histiocytic necrotizing lymphadenitis: Kikuchi-Fujimoto disease. Apropos of 6 cases].

Necrotizing Histiocytic Lymphadenitis or Kikuchi-Fujimoto disease is a benign process of lymphatic system that consists of lymphadenopathy which may be accompanied by fever, rash and some haematological alterations as anaemia. It affects usually young people, mostly women. Although it is more frequent in Oriental countries, there have been some notices about this disease in Europe recently. We present six patients that have been diagnosed in our Hospital between 1986 and 1996. All presented as cervical masses and fever. The diagnostic procedure was the lymph node biopsy, which showed the typical pathology of this disease: some foci of necrosis which contained plasmocytoides cells and atypical lymphocytes. It is also suggestive of this entity the absence of polymorphonuclear cells. Then, the diagnosis of the Necrotizing Histiocytic Lymphadenitis is made by the histology. The treatment is symptomatic. In all cases the evolution is the whole remission of symptoms. In our patients the illness was self-limited and none presented any other symptom. Our proposal with this article is to remind the clinicians of this entity when people ask for cervical masses and fever.

Adolescent↗
PubMed2003

Lack of human herpesvirus 8 and Epstein-Barr virus in Kikuchi's histiocytic necrotizing lymphadenitis.

Kikuchi's histiocytic necrotizing lymphadenitis is a self-limited disorder that typically involves the cervical lymph nodes of young women. Although a viral etiology has been postulated, a definitive viral agent has not been identified. Recent reports have suggested that human herpesvirus 8 (HHV 8) or Epstein-Barr virus (EBV) may play an etiologic role. We investigated the presence of HHV 8 and EBV in archival tissue from 34 cases of Kikuchi's histiocytic necrotizing lymphadenitis. We examined 29 cases for HHV 8 using a nested polymerase chain reaction (PCR) on paraffin-embedded or frozen tissue, and 24 cases for EBV RNA using in situ hybridization (ISH) for EBER1. Controls included reactive lymph nodes from 8 adult women presenting with cervical or axillary lymphadenopathy. The study patients included 7 men and 27 women with a mean age of 28 years. All patients were previously healthy without evidence of immunocompromise and presented with cervical, axillary, or inguinal lymphadenopathy. Two cases exhibited EBV RNA by ISH; this was confirmed by PCR for EBV DNA. HHV 8 DNA was not amplified by nested PCR in any of the cases of Kikuchi's histiocytic necrotizing lymphadenitis or reactive lymph nodes; control PCR demonstrated the presence of amplifiable DNA in all cases. These findings suggest that HHV 8 and EBV do not play causative roles in Kikuchi's histiocytic necrotizing lymphadenitis.

Adolescent↗
PubMed1991

Parvovirus B19 infection can induce histiocytic necrotizing lymphadenitis (Kikuchi's disease) associated with systemic lupus erythematosus.

We report three cases of systemic lupus erythematosus (SLE) associated with necrotizing histiocytic lymphadenitis (Kikuchi's disease) and immunologically proven human parvovirus B19 infection. Simultaneous occurrence of SLE and Kikuchi's disease was a characteristic of the three cases. Kikuchi's disease is an uncommon disease that usually affects young women and is characterized by painless unilateral cervical lymph-node enlargement. T-cell regions of affected lymph nodes are exclusively involved with patchy paracortical necrosis surrounded by a polymorphous cell population of histiocytes and macrophages. However, lymphadenopathy in patients with SLE may be histologically indistinguishable from Kikuchi's necrotizing lymphadenitis. The cause of Kikuchi's disease remains uncertain, although infectious agents have been proposed. A positive IgM-specific anti-human parvovirus B19 antibody test in our three cases suggests that B19 can induce a necrotizing histiocytic lymphadenitis and possibly a clinical SLE flare. High-dose (1 mg/kg/day) and medium-dose (0.5 mg/kg/day) oral prednisone was an effective treatment for constitutional and visceral symptoms of Kikuchi's and SLE diseases.

Adolescent↗
PubMed1989

A fatal case of multicentric Kikuchi's histiocytic necrotizing lymphadenitis.

Kikuchi's histiocytic necrotizing lymphadenitis (Kikuchi's disease) is a histologically alarming but self-limiting lesion typically affecting the cervical lymph nodes of young adults. The authors report the first case of fatality occurring during the active phase of Kikuchi's disease. The 38-year-old patient presented with fever and generalized lymphadenopathy. During hospitalization, he developed abrupt onset of heart failure and died. Postmortem examination revealed enlarged lymph nodes all over the body showing typical histologic changes of Kikuchi's disease, but there was no encroachment on vital structures. The heart was dilated and flabby, with multiple microscopic foci of necrosis and mild fatty change. There were no other significant findings, and all cultures were negative. The authors speculate that large amounts of cytokines produced by the histiocytes or high endogenous catecholamines resulting from the stress response might be responsible for the myocardial damage. Alternatively, infection by an as yet uncharacterized microbial might be the underlying cause for both the lymphadenitis and myocardial disease.

Adult↗
PubMed1991

[Histiocytic necrotizing lymphadenitis or Kikuchi and Fujimoto's disease. A case report].

We describe a case of necrotizing histiocytic lymphadenitis or Kikuchi and Fujimoto disease, a rare lymphadenopathy of unknown etiology usually striking young women. It was formerly described in Japan, and subsequently in other countries and in Italy as an anatomo-clinical entity. The disease has a favourable prognosis, therefore it has to be considered differently from other lymphadenopathies with severe prognosis whose anatomo-pathological differential features were analyzed by the authors. The case here observed differs from other cases described in literature because of the presence of a perilymphadenitic component.

Diagnosis, Differential↗
PubMed1996

[Kikuchi-Fujimoto necrotizing histiocytic lymphadenitis: apropos of 2 cases and review of the literature].

The Kikuchi-Fujimoto's syndrome is an histiocytic necrotizing lymphadenitis which is observed at any ages but preferentially among young adults. The sex ratio is about 4:1 in favour of women. It is clinically characterized by cervical lymph nodes in a context of fever and asthenia. Sometimes, there is transitory leucopenia and an increase of the erythrocyte sedimentation rate. The course of the disease is spontaneously favourable in 1 or 3 months but recurrence is possible. The histology of the lymph node could mimic a malignant lymphoma and the immunohistochemical findings are of a great importance (Ki-M1P or KP1 antibody). The etiology remains unknown but some infectious diseases have been suspected (toxoplamosis, Epstein-Barr virus). Its association with a systemic lupus erythematous had been described and this set the problem of its physiopathology. We report two new cases of Kikuchi-Fujimoto's syndrome which one was attributed to Epstein-Barr virus primo-infection and the other associated with a systemic lupus erythematous.

Adult↗
PubMedDate not supplied

Histiocytic necrotizing lymphadenitis of the neck.

Histiocytic necrotizing lymphadenitis (HNL) also known as Kikuchi or Kikuchi-Fujimato disease is a benign and self-limiting disease. The entity was first described in 1972 by Kikuchi and Fujimato in Japan independently. HNL is prevalent in Asia, although it may be seen in a wide geographic distribution. It commonly affects young women. Cervical lymphadenopathy is the most prominent symptom and should be differentiated from lymphoproliferative, autoimmune, and postinfectious diseases. Apoptosis is the main process, taking role in pathogenesis of the disease. Although it is a well-known entity among pathologists, little is written in ear, nose, and throat literature. In this study, we report 2 male patients with HNL presenting with enlarged cervical lymph nodes with a brief review of literature.

Adult↗
PubMed1997

Kikuchi disease (histiocytic necrotizing lymphadenitis) in association with HTLV1.

We report a 25-year-old male with fever, generalized lymphadenopathy and an erythematosus maculo-papular eruption affecting face and upper body. A diagnosis of Kikuchi disease (necrotizing histiocytic lymphadenitis) was made on lymph node histology. The patient was found to be positive for the human T-cell leukaemia lymphoma virus (HTLV1). Kikuchi disease has been reported in association with infections such as Epstein-Barr virus (EBV), herpes (HHV6) and toxoplasmosis, but to our knowledge this is the first case of Kikuchi disease associated with HTLV1.

Adult↗
PubMed1998

A study of the viral etiology of histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease).

Histiocytic necrotizing lymphadenitis (HNL) or Kikuchi's disease is a distinctive, self-limited disorder characterized by necrotizing cervical lymphadenopathy in young individuals. HNL is more prevalent among Asians and is a relatively common disorder among Koreans. A preceding fever, lymphopenia, and occasional skin rashes suggest a viral etiology and there have been sporadic reports of viral association. However, so far, no infectious agent has been proven to be etiologically related. In the present study, the authors examined HNL tissue samples for the presence of the genome of herpesviridae. A polymerase chain reaction was performed on 12 freshly frozen lymph nodes with HNL with a single pair of consensus primers selected within a highly conserved region of the DNA polymerase gene of the Epstein-Barr virus (EBV), designed to detect herpes simplex type 1 (HSV1), herpes simplex virus type 2 (HSV2), and cytomegalovirus (CMV) in addition to EBV. The amplified products of known sizes were then analyzed by a single restriction enzyme treatment for confirmation. No viral DNA was amplified in any of the 12 cases of histiocytic necrotizing lymphadenitis. The authors conclude that there is no evidence that HSV1, HSV2, CMV, or EBV plays any role in the pathogenesis of histiocytic necrotizing lymphadenitis.

Adolescent↗
PubMedDate not supplied

[Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease)].

INTRODUCTION: Histiocytic necrotizing lymphadenitis is a disease of unknown etiology, favourable course and outcome, mostly encountered in young women. It exhibits increased temperature and lymphadenopathy. The illness resolved spontaneously after a few months. A CASE REVIEW: In a 32-year-old patient the disease developed gradually, with pain in the right axilla, within enlarged lymph. Two weeks later, her temperature increased to 39 degrees C, accompanied by fever, shivering, malaise and loss of appetite. The ultrasonographic finding showed a conglomerate of hypoechogenic lymph nodes, with a few enlarged lymph glands. By immunohistochemical examination of a gland tissue sample the diagnosis was established Kikuchi-Fujimoto disease ("Histiocytica necrotizing lymphadenitis"--"Apoptotic lymphadenitis"). Corticosteroid therapy had been administered for one year, which resulted in both subjective improvement and regression of enlarged lymph nodes. DISCUSSION: A connection between this disease and viruses has not been proved. Presence of intracellular tubuloreticular formations suggests autoimmune etiology of histiocytic necrotizing lymphadenitis. It is considered as hyperimmune reaction of the organism induced by a viral infection. Differential diagnosis should consider taxoplasmosis, tuberculosis, lymphadenitis and malignant lymphoma. CONCLUSION: Although histiocytic necrotizing lymphadenitis is a rare disease, this case shows that it should be considered when lymphadenopathy, particularly cervical, occurs in young women, accompanied by increased temperature.

Adult↗
PubMed1988

Histiocytic necrotizing lymphadenitis.

Eleven cases of histiocytic necrotizing lymphadenitis were studied. Originally four were misdiagnosed as cervical tuberculous lymphadenitis. The characteristic symptoms and signs were local tenderness, fever, leucocytopenia and good prognosis. Antibody to Epstein-Barr virus was elevated in 2 of 3 cases. Biopsy of the affected lymph node was necessary when the differential diagnosis from lymph node tuberculosis or malignant lymphoma could not be made. The characteristic histological feature was focal necrosis without neutrophil infiltration in cortical or subcortical areas. Ultrastructurally, we found characteristic inclusions ('tubulo-reticular structures') in histiocytes and lymphocytes.

Adolescent↗
PubMedDate not supplied

Histiocytic necrotizing lymphadenitis (Kikuchi's disease).

Histiocytic necrotizing lymphadenitis (Kikuchi's disease) is a benign disorder predominantly of young women who present with cervical adenopathy. It has, on occasion, been misdiagnosed as a malignant neoplasm. Little mention of this condition has appeared in the otolaryngologic literature. A patient with Kikuchi's disease is reported and the pertinent literature is reviewed. Because both the clinical and pathological aspects of Kikuchi's disease are important in establishing the diagnosis, effective consultation between the head and neck surgeon and the pathologist is crucial.

Adult↗
PubMed1996

Involvement of cell-mediated killing in apoptosis in histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease).

Histiocytic necrotizing lymphadenitis, also called Kikuchi-Fujimoto (KF) disease, is a benign disorder characterized histologically by paracortical necrotic foci surrounded by histiocytic aggregates. We analysed affected lymph node tissues from 34 patients with the disease in an attempt to elucidate its histogenesis. The 'necrotizing' cells showed typical apoptotic changes, including cell shrinkage and condensed and fragmented nuclei. Apoptotic bodies with a peculiar ultrastructure were demonstrated, and DNA fragmentation was detected in these cells by in situ end labelling. Immunostaining for the apoptosis-regulating proteins bcl-2, bax, c-myc and p53 failed to show their involvement in KF disease. However, perforin, a killer cell-specific cytolytic protein essential for provoking apoptosis in target cells, was found to be expressed abundantly by the infiltrating cells, which were thought to be cytotoxic T-lymphocytes. Perforin-expressing cells were present in the apoptotic foci of 28 of the 34 patients (82.4%). Virtually no cells containing perforin granules were present in non-pathological regions, lymph node tissues from control subjects with reactive or tuberculous lymphadenitis or those from patients with KF disease with negligible apoptosis. Therefore, the 'necrosis' associated with KF disease appears to be attributable to trans apoptotic death of the killer cell target in the affected nodes. We propose that KF disease should be called apoptotic lymphadenitis.

Adolescent↗
PubMed1991

[Histiocytic necrotizing lymphadenitis of Kikuchi and Fujimoto].

Histiocytic necrotizing lymphadenitis of Kikuchi and Fujimoto (HNL) is a benign, self-limited disease of unknown etiology. It can easily be mistaken for lymphoma, both clinically and on pathological examination. We report a 25-year-old woman who presented with fever and painful unilateral lymphadenopathy, clinically compatible with, and histologically diagnosed as HNL. Only 2 previous cases of HNL have been reported from Israel.

Adult↗
PubMed2002

Interferon-gamma, interleukin-18, monokine induced by interferon-gamma and interferon-gamma-inducible protein-10 in histiocytic necrotizing lymphadenitis.

Apoptosis of histiocytes is a characteristic feature of necrotizing lymphadenitis (HNL). Recent studies have indicated that Fas and perforin-based pathways are involved in the apoptotic process of HNL. Elevated levels of serum interferon (IFN)-gamma are reported in HNL. The CXC chemokine interferon-gamma-inducible protein-10 (IP-10) and monokine induced by interferon-gamma (MIG) cause tissue necrosis, and interleukin (IL)-18 induces the expression of IFN-gamma and Fas ligand (FasL) by T and natural killer (NK) cells. This study was designed to determine the expression of IFN-gamma, IL-18, MIG and IP-10 in HNL. Ten cases of HNL were analyzed by using immunohistochemical staining and/or reverse transcriptase-polymerase chain reaction (RT-PCR). As a control, we included four cases of non-specific lymphadenitis. MIG and IP-10 proteins, which enhance the release of granzyme, showed a similar distribution pattern in viable tissues surrounding dead tissue, mostly within histiocytes, and lymphocytes in HNL. IL-18 was located within histiocytes, especially phagocytic histiocytes, but not within lymphocytes. In addition, IFN-gamma-positive lymphocytes were frequently detected in the surrounding dead tissue, and the lymphocytes in the same area were frequently positive for CXCR3, a specific receptor of MIG and IP-10. In non-specific lymphadenitis, MIG, IP-10 and IL-18 positive cells were detected, but their numbers were relatively small compared with HNL, while IFN-gamma positive cells were rarely encountered. Our findings suggest that the cytokine and chemokine pathways of IFN-gamma, IL-18, MIG and IP-10 play an important role in the pathogenesis of apoptosis associated with HNL.

Adolescent↗