Kikuchi Fujimoto disease: a rare case report

Kikuchi disease, also known as Kikuchi histiocyticnecrotizing lymphadenitis, was initially described in young Japanese women. It is a rare benign self-limiting disease of unknown cause usually characterized by fever and cervical lunphadenitis. It is diagnosed by doing excisional biopsy of affected lymph node. A case of young 20 years old female presented with complaint of fever and weight loss for 20 days along with cervical and axillary lymphadenopathy. Complete physical examination, radiological investigations and biochemical tests were done to rule outsystemic lupus erythematous, non-Hodgkin lymphoma and tuberculosis.Only symptomatic treatment was done along with corticosteroids. Its diagnosis is important as it can be easily mistaken for other form of lymphadenitis. Clinician and pathologists’ awareness of this disorder is very necessary.

Adenomegalia dolorosa: doença de Kikuchi entre os diagnósticos diferenciais / Painful adenomegaly: Kikuchi disease among differential diagnoses

RESUMO: A doença de Kikuchi-Fujimoto ou linfadenite histiocítica necrosante, de curso benigno, é prevalente em mulheres jovens, e associada à febre e leucopenia. Estudos recentes têm demostrado que sua etiologia ainda é incerta, sendo uma doença rara, com incidência de 0,5 a 5% de todas as adenopatias analisadas histologicamente. O diag-nóstico diferencial por imunohistoquímica foi decisivo, descartando outras hipóteses diagnósticas como: linfoma, tuberculose ganglionar e lúpus eritematoso sistêmico. Este relato de caso mostra as características da apresentação da doença em uma mulher, caucasiana e todas as etapas da investigação, destacando a importância dos diagnósticos diferenciais em adenopatias dolorosas e as dificuldades quando avaliamos portadores de doenças raras. (AU)

ABSTRACT: Kikuchi-Fujimoto disease or benign necrotizing histiocytic lymphadenitis is prevalent in young women and associated with fever and leukopenia. Recent studies have shown that its etiology is still uncertain, being a rare disease, with an incidence of 0.5 to 5% of all histologically analyzed adenopathies. Differential diagnosis by immunohistochemistry was decisive, ruling out other diagnostic hypotheses such as lymphoma, ganglion tuberculosis, and systemic lupus erythematosus. This case report shows the characteristics of the presentation of the disease in a Caucasian woman and all stages of the investigation, highlighting the importance of differential diagnoses in painful adenopathies and the difficulties when evaluating rare-disease patients. (AU)

Kikuchi's disease (histiocytic necrotizing lymphadenitis): A rare presentation with acute kidney injury, peripheral neuropathy, and aseptic meningitis with cutaneous involvement

Kikuchi's disease (KD) also known as histiocytic necrotizing lymphadenitis is rare, idiopathic, generally self-limited cause of lymphadenitis. We present a case of twenty year young female who presented in critically ill state with fever, cervical and axillary lymphadenopathy, rash, vomiting and altered sensorium and found to have neurological, hepatic, renal and dermatological involvement. Kikuchi's disease should be considered in differential diagnosis of fever and lymphadenopathy and though benign can sometimes present with multi-organ involvement. It is because of rarity of this disease with unusual complications, present case is reported.

Clinicopathological study of cervical lymphadenopathy at rural tertiary health care centre

Background: To describe the diversity of clinical manifestations, laboratory findings and outcome of chikungunya fever in patients attending SMS Hospital, Jaipur during the epidemic of 2016 (September to November).Methods: All cases of febrile illness with polyarthralgia/polyarthritis diagnosed as chikungunya were analyzed. Diagnosis was made by ELISA based IgM serology and RT PCR assay.Results: A total of 200 cases were studied. All of them presented with fever, severe crippling joint pain & tenderness, headache, anorexia and body rash. On examination, there was periarticular edema, erythema, and tenderness in joints with post auricular and cervical lymphadenopathy. Unusual manifestations were hyper pigmentation of face and forehead and scrotal ulcers. On investigations patient had leucopenia with elevated level of SGOT, SGPT with normal bilirubin levels. Other complications observed were encephalopathy, encephalitis, myocarditis and hepatitis. There was no mortality in this group.Conclusions: Chikungunya though prevalent is under-reported. The diagnostic certainty is mandated by presence of febrile transiently crippling polyarthragias / arthritis. On analyzing a large series, unusual clinical features may emerge

A Case of Kikuchi Disease Involving an Intrapulmonary Lymph Node Mistaken for a Pulmonary Nodule / 대한내과학회지

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon idiopathic, self-limiting disease characterized by cervical lymphadenopathy. Patients with KFD may present with a wide variety of nonspecific symptoms, including fever, night sweats, and weight loss. Although KFD can affect all lymph nodes of the body, involvement of the intrathoracic lymph nodes is relatively rare. In particular, isolated involvement of the intrapulmonary lymph nodes is extremely unusual. We herein report a case involving a 45-year-old man who presented with symptoms of myalgia, fatigue, and fever. Computed tomography performed during follow-up showed a slowly growing nodule in the upper lobe of the left lung. Results of laboratory tests did not reveal any evidence of infection or autoimmune disease, including systemic lupus erythematosus. Results of excisional biopsy by video-assisted thoracoscopic surgery revealed KFD in an intrapulmonary lymph node. His symptoms improved after a trial of nonsteroidal anti-inflammatory drugs.

Comparison of Ultrasonographic Findings of Biopsy-Proven Tuberculous Lymphadenitis and Kikuchi Disease

OBJECTIVE: Although tuberculous lymphadenitis and Kikuchi disease are common causes of cervical lymphadenopathy in Asians and exhibit similar clinical manifestations, their treatment strategies are totally different. The purpose of this study was to identify ultrasonographic features that distinguish these two diseases. MATERIALS AND METHODS: This study was approved by the Institutional Review Board. The study included 77 patients with tuberculous lymphadenitis and 135 patients with Kikuchi disease. The sex and age distributions of the patients were analyzed. The size and shape of lymph nodes (LNs), presence of conglomeration, increased perinodal echogenicity, echogenic hilum, posterior neck involvement, internal calcification, patterns of internal necrosis, laterality of involved LNs, and hilar vascular patterns on ultrasonography were compared between the two groups. Multiple logistic regression analysis was conducted to identify independent findings to discriminate tuberculous lymphadenitis from Kikuchi disease. Finally, diagnostic accuracies were calculated using the independent findings. RESULTS: The presence of an echogenic hilum, internal calcification, patterns of internal necrosis, and LN hilar vascular structures on power Doppler ultrasonography were independent findings that discriminated tuberculous lymphadenitis from Kikuchi disease. The diagnostic accuracy of each of these four factors was 84.9% (181/212), 76.9% (163/212), 84% (178/212), and 89.2% (189/212), respectively. A combination of internal calcification and hilar vascular structures showed the best accuracy of 89.6% (190/212) (sensitivity, 86.7% [117/135]; specificity, 94.8% [73/77]) for diagnosing Kikuchi disease. CONCLUSION: The presence of an echogenic hilum, internal calcification, pattern of internal necrosis, and LN hilar vascular structures are useful ultrasonographic findings to differentiate tuberculous lymphadenitis from Kikuchi disease.

Cervical Lymphadenopathy—Pitfalls of Blind Antitubercular Treatment.

Tuberculosis (TB) is the most common cause of cervical lymphadenopathy in the TB-endemic zone, like India but it can also mimic other diseases. Four cases of cervical lymphadenopathy presented to us as initial treatment failure after completion of six months of antitubercular drugs (ATD), including rifampicin, isoniazid, pyrazinamide, and ethambutol. All were diagnosed as having tuberculosis either by fine needle aspiration cytology or clinically from outside our institution. In one case, tuberculosis was the final diagnosis but, unfortunately, it was multidrug-resistant. In other three cases, Hodgkin disease, Non-Hodgkin lymphoma, and Kikuchi’s disease were the diagnoses. In resource-poor countries, like India, which is also a TB-endemic zone, TB should be the first diagnosis in all cases of chronic cervical lymphadenopathy, based on clinical and/or cytological evidences. So, they were correctly advised antitubercular therapy (ATT) initially. Sometimes, TB mimics other aetiologies where apparent initial improvement with ATT finally results in treatment failure. Hence, investigations for microbiological and histopathological diagnosis are warranted, depending on the resources and feasibility. If these tests are not routinely available, the patients should be under close monitoring so that lymphoma, drug-resistant TB, or other aetiologies of cervical lymphadenopathy are not missed. Patients with cervical lymphadenopathy rarely presents acutely; so, a physician can take the opportunity of histopathological study of lymphnode tissue.

Tuberculosis ganglionar cervical simulando una enfermedad de Kikuchi: reporte de un caso y revisión de la literatura / Cervical lymph node tuberculosis simulating Kikuchi's disease: a case report and review of the literature

La linfadenitis cervical tuberculosa es la manifestación más común de tuberculosis en cabeza y cuello. La enfermedad de Kikuchi es una enfermedad rara que consiste en una linfadenitis necrotizante histocitiaria. La presentación clínica en ambas entidades suele consistir en una o más masas cervicales dolorosas, que pueden ser duras o fluctuantes. Los hallazgos por imagen no permiten distinguir de manera absoluta la linfadenitis tuberculosa de la enfermedad de Kikuchi. Por lo tanto es imprescindible para interpretar los hallazgos por imagen no sólo conocer el origen demográfico del paciente, sino su estado inmunológico con respecto a la tuberculosis y el estudio histopatológico y microbiológico de las adenopatías.

Tuberculous cervical lymphadenitis is the most common manifestation of tuberculosis in the head and neck. Kikuchi's disease is a rare disease that is histocitiaria necrotizing lymphadenitis. The clinical presentation in both entities is usually a painful neck masses or more, which can be hard or fluctuating. The imaging findings do not distinguish in absolute tuberculous lymphadenitis Kikuchi's disease. Therefore it is essential to interpret the imaging findings not only know the origin of the patient population, but their immune status with regard to Tuberculosis and histopathological and microbiological lymphadenopathy.

Histiocytic necrotising lymphadenitis (kikuchi's disease) [a 5 year histomorphological study].

Background: Kikuchi’s disease is a rare, benign, self-limiting disease, mainly involving the lymph nodes of young people. The disease has unknown etiology. Clinical symptoms and basic investigations in a patient often leads to a misdiagnosis, due to similar findings in other lesions like, lymphomas and chronic granulomatous inflammations of lymph nodes like tuberculosis. Lymph node biopsy shows characteristic diagnostic features. Methods: We studied all lymph node biopsies received in department of pathology & from other private laboratories in Rural and Urban areas, in & around Bareilly region of North India, in 5 years (2006-2010). Relevant clinical data was collected. Findings were correlated with previous studies. A total number of 1015 cases were examined. Results: A total of 15 cases showed histopathological features of Kikuchi’s disease. Most number of cases was seen in females, in the age group of 20-25 years. Patients presented with fever and lymphadenopathy, with a clinical provisional diagnosis of tuberculosis in maximum number of cases.Conclusion: Necrotising lymphadenitis has a striking predilection for cervical lymph nodes of females and is usually accompanied by fever. Most commonly, this disease is mistaken for tuberculosis or Lupus Lymphadenitis. Detailed clinical examination and characteristic histopathological findings help in diagnosing these lesions.

A Patient with Kikuchi's Disease: What Should Pain Clinicians Do?

Kikuchi's disease (KD) is an idiopathic and self-limiting necrotizing lymphadenitis that predominantly occurs in young females. It is common in Asia, and the cervical lymph nodes are commonly involved. Generally, KD has symptoms and signs of lymph node tenderness, fever, and leukocytopenia, but there are no reports on treatment for the associated myofacial pain. We herein report a young female patient who visited a pain clinic and received a trigger point injection 2 weeks before the diagnosis of KD. When young female patients with myofascial pain visit a pain clinic, doctors should be concerned about the possibility of KD, which is rare but can cause severe complications.

Lupus Erythematosus Profundus Associated with Kikuchi's Disease / 대한피부과학회지

Kikuchi's disease (KD), histiocytic necrotizing lymphadenitis, is a rare self-limited lymphadenopathy, which usually affects young women. KD has been reported to precede, coexist with or follow the diagnosis of other entities, such as systemic lupus erythematosus (SLE), adult-onset Still's disease, Hashimoto's disease, and viral infections. In a few cases, KD is associated with cutaneous lupus erythematosus (CLE), without systemic involvement. Herein, we report the first Korean case of KD associated with lupus erythematous profundus in a 9-year-old boy.

A Case of Kikuchi Disease Accompanied with Bilateral Retinal Vasculitis

Kikuchi disease, also called histiocytic necrotizing lymphadenitis, is an uncommon, idiopathic and generally self-limited disease, characterized by cervical lymphadenopathy. It can present systemic symptoms and signs, but ocular involvement is unusual. We report a 35-year-old woman who presented sudden decreased visual acuity and a swollen lymph node on the left side of her neck. On laboratory findings, there were no evidences of infection, autoimmune disease and systemic vasculitis. She was diagnosed with Kikuchi disease and bilateral retinal vasculitis by histologic analysis of lymph node, fundoscopy and fluorescein angiography.

Kikuchi-Fujimoto disease: three case reports / Doença de Kikuchi-Fujimoto: relato de três casos

CONTEXT: Kikuchi-Fujimoto disease (KFD) manifests in most cases as unilateral cervical lymphadenomegaly, with or without accompanying fever. The disease mainly affects young women and has a self-limited course. It is more common in oriental countries, with few reports of its occurrence in Brazil. KFD should be included in the differential diagnosis of suspected cases of viral infections, tuberculosis, reactive lymphadenitis, systemic lupus erythematosus and metastatic diseases. It can be histologically confused with lymphoma. The disease is benign and self-limiting and an excisional biopsy of an affected lymph node is necessary for diagnosis. There is no specific therapy. CASE REPORTS: This study reports on three cases of non-Asian female patients with KFD who were attended at our service between 2003 and 2006. A review of the literature was carried out, with a systematic search on this topic, with the aim of informing physicians about this entity that is manifested by cervical masses and fever.

CONTEXTO: A doença de Kikuchi-Fujimoto (DKF) se manifesta na maioria das vezes como linfadenomegalia cervical unilateral, que pode ser acompanhada de febre. Afeta principalmente mulheres jovens, com evolução autolimitada. A prevalência desta doença é mais comum no oriente, havendo poucos relatos de sua ocorrência em nosso meio. DKF deve ser incluída no diagnóstico diferencial de casos suspeitos de infecções por vírus, tuberculose, linfadenite reacional, lúpus eritematoso sistêmico e doença metastática. Histologicamente pode ser confundida com linfoma. A doença é de caráter benigno, auto-limitada, a biópsia excisional do linfonodo acometido é necessária para o diagnóstico e não há tratamento específico. RELATO DE CASOS: Este trabalho relata três casos de pacientes não asiáticas do sexo feminino com DKF, atendidas em nosso serviço entre 2003 e 2006, e faz revisão da literatura com uma busca sistematizada sobre o assunto, com o objetivo de informar os médicos sobre essa entidade que se manifesta com massa cervical e febre.

A Case of Kikuchi's Disease Presenting with Unique Facial Involvement

Kikuchi's disease (KD), or histiocytic necrotizing lymphadenitis, is a rare, self-limited lymphadenopathy, typically in young women, that usually remits spontaneously and does not recur. KD is clinically characterized by cervical lymphadenopathy and a high fever. Extranodal involvement (skin, arthritis, meningitis) rarely occurs. When KD is involved in a skin lesion, it presents with various shapes, but rarely manifests with symmetrically distributed, erythematous, firm nodules only on the face. In this report, we describe a patient with KD and unique skin manifestations.

Kikuchi's Disease Presented with Cutaneous Lesion Showing Diffuse Erythematous Maculopatches / 대한피부과학회지

Kikuchi's disease, or histiocytic necrotizing lymphadenopathy is an idiopathic, benign self-limiting lymphadenitis usually affecting young women. Cutaneous findings in Kikuchi's disease have been reported in 16~40% of cases. A 29-year-old woman presented with pruritic diffuse erythematous maculopatches on whole body accompanied by long-lasting fever and cervical lymphadenopathy. Skin biopsy showed vacuolar degeneration of the basal layer and dermal infiltration of CD68-positive histiocytes. Lymph node fine needle aspiration specimens showed abundant lymphocytes, histiocytes, karyorrhectic debris without neutrophils, consistent with Kikuchi's disease. We present a case of Kikuchi's disease showing generalized skin eruption with a review of literature that describes patterns of skin involvement.

A Case of Kikuchi Fujimoto's Disease Accompanied by Hemophagocytic Lymphohistiocytosis / 대한혈액학회지

Kikuchi's disease and hemophagocytic lymphohistiocytosis (HLH) present different clinical characteristics, especially in prognosis, although both diseases have the clinical similarity in initial presentations. Kikuchi's disease usually has a self-limiting clinical course, but HLH can be fatal. Accordingly, it is important that the differential diagnoses and decision as to initial treatment be made as soon as possible, at the time of clinical presentation. In the case of Kikuchi's disease accompanied with HLH, the decision concerning initial treatment can be very difficult, because these cases have been rarely reported and the prognosis is unpredictable. We report a case of a 21-year-old female diagnosed with Kikuchi's disease accompanied with HLH. Treatment involved steroid therapy, as for treatment of HLH. Recovery was complete. Kikuchi's disease with HLH can be completely treated with more aggressive therapy than used for Kikuchi's disease alone.

Intra-abdominal Kikuchi's Disease Mimicking Malignant Lymphoma on FDG PET-CT / 핵의학분자영상

Kikuchi's disease is a self-limiting benign disease characterized by cervical lymphadenopathy, but it can be mistaken for malignant disease, and when involved lymph nodes are unusually located, diagnosis can be more difficult. The authors report the case of a 19-year-old man with Kikuchi's disease, who had isolated intraabdominal lymphadenopathy and increased 18-fluoro-deoxyglucose (FDG) uptake in positron emission tomographycomputed tomography (PET-CT). Although its incidence is extremely rare, intra-abdominal Kikuchi's disease with increased FDG uptake in PET-CT image should be considered in the differential diagnosis when constitutional symptoms mimic those of malignant lymphoma.

A Case of Kikuchi's Histiocytic Necrotizing Lymphadenitis Associated with Systemic Lupus Erythemasus / 대한피부과학회지

Kikuchi's disease (KD), histiocytic necrotizing lymphadenitis, isa benign and self-limited disease which mainly affects young women. KD has been rarely described in association with systemic lupus erythematosus (SLE), and its diagnosis can precede, postdate or coincide with the diagnosis of SLE. The tendency of flare-up of lupus activity with the onset of KD and the simultaneous occurrence of these two diseases suggest that they are not independent events. Herein, we report a case of KD associated with SLE in an 18-year-old female who showed tender erythematous papules on both upper extremities accompanied by cervical lymphadenopathy. The histopathologic examination of lymph nodes showed histiocytic necrotizing lymphadenitis.

Acute Tonsillitis With Concurrent Kikuchi’s Disease as a Cause of Persistent Lymphadenopathy

We present a young adult female with symptoms of acute tonsillitis and tender cervical lymphadenopathy. Despite a full course of oral antibiotics, she had persistent left lower cervical lymphadenopathy measuring 2.0 x 1.5 cm at 2 weeks post-treatment. Rigid and flexible scope examinations did not reveal any abnormalities in the nasopharynx, oropharynx or hypopharynx. Tuberculosis tests were negative and blood index results were normal. Fine needle aspiration cytology revealed a non-specific granulomatous inflammatory process. Excisional lymph node biopsy was performed, and the patient was diagnosed as having Kikuchi’s Disease (KD). We would like to highlight the diagnostic challenges in detecting this condition and the importance of differentiating KD from tuberculosis and malignant lymphoma, the latter of which requires aggressive treatment.