Kikuchi's disease as a presenting feature of mixed connective tissue disease

Summary Necrotising histiocytic lymphadenitis (Kikuchi's disease) is a recognised cause of benign lymphadenopathy. It has been reported in association with both adult Still's disease, systemic lupus erythematosus (SLE), and mixed connective tissue disease (MCTD). We report a case of mixed connective tissue disease (MCTD), in which biopsy of enlarged lymph nodes occurring as a presenting feature showed the characteristic features of Kikuchi's disease. This finding supports the belief that Kikuchi's disease and the autoimmune rheumatic disorders may share a common aetiology.     

Pathogenic links between Kikuchi's disease and lupus: a report of three new cases

INTRODUCTION: Histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto's disease is a rare anatomoclinical entity whose etiology remains unknown. It is mainly reported in young adult female, presenting with cervical lymphadenopathies, fever and asthenia. The diagnosis is based on the histological examination of a lymph node biopsy. The disease course is usually uneventful, but sometimes Kikuchi-Fujimoto's disease can reveal or evolve into a cutaneous or a systemic lupus. EXEGESIS: We report three new cases of Kikuchi's disease: the first one mimicked a systemic lupus, the second one was associated with a lupus-like rash, and a the last one was a severe case with hemophagocytic syndrome and a primo-infection with Epstein-Barr virus revealing a systemic lupus erythematosus. CONCLUSION: Clinical and biological follow-up of patients presenting with Kikuchi's disease is necessary to look for an association with a lupus. We discuss the pathogenic links between Kikuchi's disease and lupus.     

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.     

Kikuchi's disease in systemic lupus erythematosus: an independent or dependent event?

Summary The authors describe necrotizing histiocytic lymphadenitis (Kikuchi's disease) in association with systemic lupus erythematosus (SLE). To our knowledge this is the first case report where SLE preceded Kikuchi's disease. Whether Kikuchi's disease is an independent event or directly connected with SLE is discussed.     

Three clinical reports of Kikuchi's lymphadenitis combined with systemic lupus erythematosus

Summary The authors describe three cases of systemic lupus erythematosus (SLE) associated with Kikuchi's histiocitic necrotizing lymphadenitis (HNL). Two patients presented a cytomegalovirus infection concomitantly with Kikuchi's lymphadenitis; in one of them the onsets of SLE and HNL were simultaneous. In the third case an inguinal HNL was concomitant with vaginitis of unknown aetiology.     

Kikuchi-Fujimoto's disease and connective tissue disease: a report of three cases

INTRODUCTION: Kikuchi-Fujimoto's disease or histiocytic necrotizing lymphadenitis, clinicopathological entity of unknown aetiology, is a rare and benign cause of cervical lymphadenopathies. It can be associated with various auto-immune diseases especially systemic lupus erythematous (SLE) or with some infectious agents. EXEGESIS: This report describes a survey of three patients who developed Kikuchi's lymphadenitis occurring concomitantly with connective tissue disease: LES in two cases and non determined connective tissue disease in the other case. Comparing the clinical, histopathological and evolutionary findings to the literature allows to identify the main features of this self-limiting disorder: occurrence in young women; clinical presentation with cervical lymphadenopathy in a context of fever and asthenia. The definite diagnosis is usually made through histopathological examination of a lymph node biopsy. Disease course is generally favourable with spontaneous resolution within few weeks. It may be improved with corticosteroid treatment in patients with systemic involvement. Prognosis is related to the associated disease. CONCLUSION: Kikuchi-Fujimoto's disease is a rare and benign cause of cervical lymphadenopathy that could resemble lymphoma, tuberculosis and may be associated with a characterized systemic disease.     

Systemic juvenile idiopathic arthritis,Kikuchi's disease and haemophagocytic lymphohistiocytosis--is there a link? Case report and literature review

SIR, Necrotizing lymphadenitis (Kikuchi's disease) is a benigncervical lymphadenopathy that typically affects young womenand usually remits spontaneously with no recurrences [1]. Histologyof involved lymph nodes shows a characteristic picture [2–6].Systemic juvenile idiopathic arthritis (JIA) accounts for 10–20%of JIA, affecting males and females equally and occurring mostfrequently under the age of 5 yr. Generalized lymphadenopathyis found in 70–80% of cases [7, 8]. There are very fewreports in the literature of systemic JIA being associated withKikuchi's disease. One report describes three cases of adult-onsetStill's disease associated with Kikuchi's disease [9]. Thereis only one report in children and     

What is your diagnosis?: SLE and Kikuchi's disease: A rare association

Kikuchi–Fujimoto disease (KFD), or histiocytic necrotising lymphadenitis, is a rare benign, self-limiting cervical lymphadenitis of unknown aetiology. It predominantly affects young women and can closely mimic infective and immunological disorders. There is also a rare but documented association with systemic lupus erythematosus (SLE). We present the case of a young woman with biopsy-proven KD who was also diagnosed with SLE.     

Kikuchi's disease: an uncommon cause of fever of unknown origin

Kikuchi's disease or histiocytic necrotizing lymphadenitis is an uncommon clinical condition that causes diagnostic difficulties because of its lack of specific signs, symptoms and serological markers. Diagnosis is based on histopathological findings, but overlapping of the histological features requires a differential diagnosis among histiocytic necrotizing lymphadenitis and a number of infectious, autoimmune and lymphoproliferative lymphadenopathies. We report a case of Kikuchi's disease in a 19-year-old woman presenting with generalized lymphadenopathy and persistent fever.     

Still's disease associated with necrotizing lymphadenitis (Kikuchi's disease): report of 3 cases

Necrotizing lymphadenitis (Kikuchi's disease) is recognized as one of the benign lymphadenopathies. Previously, this condition has not been described in Still's disease which is frequently associated with lymphadenopathy. We report 3 cases of Still's disease, 2 in adults and 1 in an older child, with the findings of necrotizing lymphadenitis on lymph node biopsy. Considering the common infectious and/or immunologic etiologies described in both disorders, coexistence of both conditions suggests the possibility that they share a common etiology.     

Parvovirus B19-associated haemophagocytic syndrome with lymphadenopathy resembling histiocytic necrotizing lymphadenitis (Kikuchi's disease)

A 15-year-old girl developed a haemophagocytic syndrome caused by human parvovirus B19 (PVB19). The cervical lymph node histology, resembling that of histiocytic necrotizing lymphadenitis (HNL, Kikuchi's disease), included several transformed lymphocytes, numerous histiocytes, and massive necrosis. We detected PVB19-positive cells in the lymph node by immunohistochemistry. Possible autoimmune mechanisms in HNL-like diseases triggered by PVB19 are discussed.     

Kikuchi's disease accompanied by lupus-like butterfly rash

Kikuchi's disease (KD) is a benign and self-limiting lymphadenitis, particularly affecting young women. KD is often associated with fever, arthralgia, and leukopenia, features also found in systemic lupus erythematosus (SLE). Lymphadenitis associated with SLE is indistinguishable from that in KD, and the association of KD and SLE has been previously reported. We describe a case of KD who developed a typical butterfly rash, reminiscent of SLE. However, histological and laboratory findings excluded the diagnosis of SLE. This case emphasizes that careful differential diagnosis between KD and SLE is required.     

Therapeutic response and long-term follow-up in a systemic lupus erythematosus patient presenting with Kikuchi's disease

Kikuchi's disease (KD) can occur in association with systemic lupus erythematosus (SLE). The treatment of concomitant diseases, however, is unclear. We describe a case of a 45-y-old man who presented with generalized histiocytic necrotizing lymphadenitis, fever, malaise and weight loss. Ten months later he also developed arthritis, serositis, anemia, leukopenia and lymphopenia. ANA, anti-Smith, anti-snRNP and anti-Ro antibodies were positive. He responded rapidly and favorably to mid-dose prednisone. Hydroxychloroquine, added 5 months later, allowed tapering down and discontinuation of prednisone treatment. He has remained in complete remission for 5 years.     

Kikuchi-Fujimoto's disease and the association with systemic lupus erythematosus

Kikuchi-Fujimoto's disease (KFD), Histiocytic Necrotizing lymphadenitis, is a rare self-limiting condition characterized by lymphadenopathy, fever and neutropenia. The aetiology of KFD is controversial, and its diagnosis is confirmed histologically. Although KFD has rarely been reported associated to Systemic lupus erythemotosus (SLE) should be ruled out given its different prognosis and management. We present the clinical, histological and evolution the two cases of patients with Kikuchi's disease; one case had evolution classic and the other case were associated with SLE.     

All necrotizing nodes are not tuberculosis – A report of two cases

IntroductionKikuchi-Fujimoto disease is a benign and self-limiting systemic disorder of unknown aetiology characterised by fever, superficial lymphadenopathy and leukopenia. In highly endemic & low-resource country like India, it is frequently misdiagnosed as tuberculosis.Case reportBoth the cases were diagnosed as necrotizing lymphadenitis by fine-needle aspiration cytology. Tuberculin skin prick test (TST) was positive for one case and negative for the other case. Cartridge based nucleic acid amplification test (CBNAAT) from lymph node aspirate was negative for mycobacterium tuberculosis in both the cases, later on histopathology of lymph node showed diagnosis of Kikuchi-Fujimoto disease.ConclusionKikuchi Fujimoto is a self-limiting disease systemic disease of unknown aetiology. A definite diagnosis can be established by incisional/excisional biopsy of the lymph node. When dealing with cases of tubercular lymphadenitis, Kikuchi-Fujimoto disease should be kept as differential diagnosis.     

Nosocomial Kikuchi's Disease – A Search for Herpesvirus Sequences in Lymph Node Tissues Using PCR

Background: Histiocytic necrotizing lymphadenitis, also known as Kikuchi's disease (KD), is a rare disease. Fever and lymphadenopathies with characteristic pathologic features are present. The etiology of this disease remains undetermined. Since the disorder is self-limiting, different viruses have been implicated as the causative agent. Patients and Methods: Seven cases of KD were studied. Three patients acquired the disease nosocomially, three had community-acquired KD and one case was associated with systemic lupus erythematosus. PCR was performed on DNA extracted from lymph node tissues in order to detect herpesvirus-specific DNA sequences: herpes simplex virus type 1 and 2 (HSV 1–2), varicella zoster virus (VZV), human cytomegalovirus (HCM), human herpesvirus 6 (HHV6), Epstein-Barr virus (EBV) and human herpesvirus 8 (HHV8). Results: Viral DNA was not detected in any of the lymph node tissues from the seven cases of KD. Conclusion: We conclude that these herpesviruses were not involved in the etiology of the three cases of nosocomial KD nor in the other four cases of KD investigated in this study. Received: August 16, 2000 · Revision accepted: February 28, 2001     

Kikuchi's lymphadenitis: report of a Yersinia enterocolitica-associated case and an overview of aetiology and clinical outcome.

Kikuchi's lymphadenitis (KL; histiocytic necrotizing lymphadenitis without granulocytic infiltration) is a generally benign, febrile disorder of unknown aetiology with distinct histological features. To date, a minority of cases reported have been associated with infectious agents. A typical pathological case of KL is described where involvement of Yersinia enterocolitica was shown by an indirect immunofluorescent assay applied to lymphatic tissue. The case is discussed with emphasis on recent insight into the course and aetiology of KL.     

Mycobacterium szulgai lymphadenitis mimicking Kikuchi's disease in Thailand

Mycobacterium szulgai is rarely found to be a cause of reported infection. Two thirds of cases were reported as pulmonary presentations, while the rest were infections of soft tissues or bone. In Thailand, few pulmonary infections due to M. szulgai have been reported. This is the first case of M. szulgai lymphadenitis confirmed by tissue culture. The patient presented with prolonged fever and tender enlarged cervical nodes. Histological findings showed large histiocytes with necrotic background compatible with Kikuchi's disease. However, the culture proved the case to be one of M. szulgai infection. That means this mycobacterium should be included in the differential diagnosis of fever with lymphadenitis.     

Kikuchi's disease and systemic lupus erythematosus: case report and review

The case of a 50-year-old male with a clinical syndrome consistent with systemic lupus erythematosus (SLE), generalized lymphadenopathy, and renal involvement is presented. The pathologic examination of an excised supraclavicular lymph node showed necrotizing lymphadenitis. Although necrotizing lymphadenitis is a well-known characteristic of SLE lymphadenitis, the main histologic and immunophenotypic findings of our case showed the presence of distinctive Kikuchi's lymphadenitis.