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Kikuchi-Fujimoto Disease

Kikuchi Disease


Presentation

  • Abstract Kikuchi Fujimoto Disease (KFD) can present with dysphasia, fever and lymphadenopathy. A young Bangladeshi girl presented with fever, cervical lymphadenopathy, dysphasia, weight loss and skin rash.[ncbi.nlm.nih.gov]
Axillary Lymphadenopathy
  • We describe a case which presented with thrombocytopenia and axillary lymphadenopathy in addition to the usual features.[ncbi.nlm.nih.gov]
Mediastinal Lymphadenopathy
  • We report an unusual presentation in a South Asian woman with isolated mediastinal lymphadenopathy masquerading as tuberculosis. There is only one other reported case in the literature presenting with isolated mediastinal lymphadenopathy.[ncbi.nlm.nih.gov]
  • Chest skiagram [Figure 2] showed the presence of mediastinal widening suggestive of mediastinal lymphadenopathy. Ultrasound examination of the abdomen showed the presence of periportal, peripancreatic and paraaortic lymphadenopathy.[ijhas.in]
Asymptomatic
  • Patient's lymphadenopathy had almost resolved and he was completely asymptomatic after three months. In patient with cervical lymphadenopathy KFD should be considered in the differential diagnosis.[ncbi.nlm.nih.gov]
  • On review at 1 month, the patient’s lymphadenopathy had almost resolved and at 3 months he was completely asymptomatic. All physical and laboratory examination were in normal limit.[exp-oncology.com.ua]
  • Within 6 months follow up, he was asymptomatic. He does not show any features suggestive of SLE and /or recurrence.[casereports.in]
Raynaud Phenomenon
  • We present a case of a young Pakistani boy who presented with cervical lymphadenopathy, fever, blackish discoloration of finger tips, and Raynaud's phenomenon. His lymph node biopsy was suggestive of KFD.[ncbi.nlm.nih.gov]
Pleural Effusion
  • Some days after admission the patient got worse, he developed generalized lymphadenopathy, bilateral pleural effusion and interstitial lung disease.[ncbi.nlm.nih.gov]
Left Lower Quadrant Pain
  • A 19-year-old man was brought to our clinic with complaints of fever, headache, fatigue, and left lower quadrant pain that had persisted for 3 weeks. On physical examination, painful cervical lymphadenopathies were observed.[ncbi.nlm.nih.gov]
Constipation
  • A 12-year-old boy presented with a 1-month history of fever, abdominal pain, constipation and weight loss, and a painful lymph node was detected in the right axilla. Chest CT demonstrated multiple lymph nodes, especially in the left mediastinum.[ncbi.nlm.nih.gov]
Thrombosis
  • Anticoagulant treatment for the venous thrombosis was initiated. As KFD is often associated with a transition to systemic lupus erythematosus follow up visits are scheduled in our outpatient clinic.[ncbi.nlm.nih.gov]
Neck Pain
  • We report a 35-year-old female who presented with soft to firm cervical lymphadenopathy and neck pain. She had multiple enlarged cervical nodes. Examination of other systems was normal.[ncbi.nlm.nih.gov]
  • A 30-year-old young female presented 2 months post-partum with complaints of neck pain and fever with CT scan showing enlarged right- sided lymph nodes with a thyroid nodule.[slideplayer.com]
Cotton Wool Spots
  • Extensive cotton wool spots were observed in the right fundus, and retinal capillary occlusions were detected by OCT angiography of the left eye.[ncbi.nlm.nih.gov]
Progressive Loss of Vision
  • We report the case of a 30-year-old Asian woman who presented a rapidly progressive loss of vision.[ncbi.nlm.nih.gov]
Lethargy
  • A 29-year-old British Pakistani woman presented with a 2-month history of drenching fevers, night sweats, lethargy and tender cervical and axillary lymphadenopathy. Initial investigations, bloods and imaging were unremarkable.[ncbi.nlm.nih.gov]
  • It typically manifests as a lymphadenopathy (predominantly cervical) with constitutional symptoms like fever, lethargy and myalgia.[casereports.in]
Dysphasia
  • Abstract Kikuchi Fujimoto Disease (KFD) can present with dysphasia, fever and lymphadenopathy. A young Bangladeshi girl presented with fever, cervical lymphadenopathy, dysphasia, weight loss and skin rash.[ncbi.nlm.nih.gov]
Peripheral Neuropathy
  • We present a 14-year-old boy with a severe form of Kikuchi-Fujimoto disease, complicated by peripheral neuropathy. This patient is interesting for both his age and his peculiar complication.[ncbi.nlm.nih.gov]
Neurologic Manifestation
  • Neurological manifestations have been reported in Kikuchi-Fujimoto disease (KFD). Characteristics of brain lesions are not defined. In addition, no biological indexes are known to help clinicians along the diagnosis process.[ncbi.nlm.nih.gov]

Workup

  • A complete workup of infectious, rheumatologic, and neoplastic diseases was pursued. Lymph node biopsies revealed histiocyte proliferation with areas of necrosis. These findings were diagnostic of KFD.[ncbi.nlm.nih.gov]
Bicytopenia
  • Laboratory analysis showed bicytopenia, and radiological studies showed systemic lymphadenopathy accompanied by splenomegaly. A bone marrow examination showed hemophagocytic macrophages, suggesting HLH.[ncbi.nlm.nih.gov]
Cytopenia
  • 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.[ncbi.nlm.nih.gov]
Pleural Effusion
  • Some days after admission the patient got worse, he developed generalized lymphadenopathy, bilateral pleural effusion and interstitial lung disease.[ncbi.nlm.nih.gov]

Treatment

  • Awareness of this disorder will help prevent misdiagnosis and inappropriate treatment.[ncbi.nlm.nih.gov]

Prognosis

  • A long-term prognosis remains uncertain, and careful follow-up is preferred.[ncbi.nlm.nih.gov]
  • A review of CT neck findings in patients with Kikuchi disease (n 96) showed 1 : homogenous nodal enlargement (83%) perinodal infiltration (80%) central nodal necrotic change (16%) Treatment and prognosis It usually runs a benign course that is self-limiting[radiopaedia.org]
  • Early corticosteroid therapy may improve the long-term prognosis of KFD in children.[orca.cf.ac.uk]

Etiology

  • However, the association between human herpesvirus 7 and Kikuchi's disease has been rarely reported as a possible etiologic agent of Kikuchi's disease.[ncbi.nlm.nih.gov]

Epidemiology

  • CONCLUSION: We report the first epidemiologic description of KFD in a population of sub-Saharan origin.[ncbi.nlm.nih.gov]
  • Conclusion: We report here the first KFD epidemiology.[acrabstracts.org]
  • Epidemiology [ edit ] Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting disorder that typically affects the cervical lymph nodes.[en.wikipedia.org]
Sex distribution
Age distribution

Pathophysiology

  • Department of Otolaryngology Head and Neck Surgery , Himeji Red Cross Hospital , Hyogo , Japan ; 4 d Department of Pathology , Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences , Okayama , Japan ; 5 e Division of Pathophysiology[ncbi.nlm.nih.gov]
  • Pathophysiology [ edit ] Some studies have suggested a genetic predisposition to the proposed autoimmune response. Several infectious candidates have been associated with Kikuchi disease. [8] Many theories exist about the cause of KFD.[en.wikipedia.org]

Prevention

  • Awareness of this disorder by clinicians and pathologists will help prevent misdiagnosis and inappropriate treatment.[ncbi.nlm.nih.gov]

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