Thus more specific analyses must be carried out to avoid misdiagnosis and mistreatment. Pathological analysis of the lymph node biopsy is frequently performed. The diseased tissues generally show extensive coagulative necrosis and histiocytosis in the cortical and paracortical regions of the lymph nodes see Fig. The histological features, such as clusters of plasma-like mononuclear cells with scattered nuclear debris and crescent-shaped tissue cells, are indistinguishable from those of lymphoma.
So additional diagnostic procedures are required. For example, as immunohistochemical staining can reveal the presence of MPO-positive and CDpositive cells [ 7 ], such a pathological tool would be useful for both diagnostic and differential diagnosis. In comparison with pathological analysis such as biopsy, medical imaging is much quicker and less invasive. Magnetic resonance image and ultrasound scan have been used in our case to confirm lymph node enlargement.
As discussed above, the main symptoms for KFD are un-explained fever and lymphadenopathy. Thus, the following observations are particularly worth noting: a long-term fever even after antibiotic treatment; b large superficial lymph nodes, but little or no sign of hepatosplenomegaly and c negative results from blood test and bone marrow culture. Due to non-specific clinical manifestations, the disease can be misdiagnosed as other disorders, such as tumors e.
TbL or cat scratch disease [ 18 ] or concurrent systemic lupus erythematosus SLE. A comparison of KFD with some related disorders is shown in Table 2 , listing different characteristics for each of the disorders. Table 2 shows that fever exists in all these listed disorders, so cannot be used to distinguish KFD from others. However, detailed temperature charts may be helpful.
Although pain is felt in some of the listed disorders, but not always complained of by the patients. The sensation of pain is rather subjective and thus cannot be used for differential diagnosis.
The self-limiting factor is well noteworthy during the management of the symptoms, however, it is no practical use in terms of diagnosis. Although histopathological features are not always the same among all these disorders, they do display certain characteristics useful for diagnosis. KFD and NHL share certain histopathological features, such as proliferation of immunoblasts and plasmacytoid dendritic cells at the edges of necrotic foci [ 20 ].
Immuno-staining would be helpful as the positivity of histiocytes for myeloperoxidase can be used to exclude T-cell lymphomas and can offer useful clues regarding infectious agents. For instance, the positivity of PPD test can certainly consolidate the diagnosis of tuberculosis while the finding of Bartonella henselae by Warthin—Starry stain would almost certainly confirm the case for CSD as illustrated in Fig. KFD: Kikuchi-Fujimoto disease.
Clearly, the diagnosis of KFD can be difficult and its differential diagnosis is more demanding. To aid medical practitioners, in particular, non-specialists to identify this rare disorder, a diagnostic flow chat is proposed in Fig.
Once a cervical lymphadenopathy is presented, appropriate medical assessments should be carried out. Physical examinations and imaging techniques can be used to rule out certain forms of lymphadenitis e. CSD and TbL. Clinically, patients with unexplained fever should be carefully examined and closely monitored regarding any change with lymph nodes. If no improvement is observed in a week or so after the treatment with antibiotics, medical professionals should consider the possibility of KFD by conducting a lymph node biopsy to determine pathological features.
Once KFD is confirmed and malignant lymphoma or other lymphatic diseases such as TbL or CSD are ruled out, appropriate steps are taken to facilitate the relief of the symptoms. There is no universally agreed treatment plan as each case could be somehow different. The primary treatment of KFD is to manage the disease by supporting the patient mentally and physically to speed-up the relief of the symptoms. Table 1 lists some successful treatments to relieve the KFD symptoms.
Antibiotics are not effective, however, their use in this case [ 13 ] is to avoid potential bacterial infections. The use of hormone-related drugs can relieve the symptoms and shorten the course of the disease.
Prednisone was used as an effective treatment for pregnant women [ 9 ]. Generally, KFD is a benign and self-limiting disorder, so a short inpatient stay with symptomatic treatment as in our case generally suffice. However, for patients with significant systemic symptoms e. In summary, Kikuchi-Fujimoto disease is a rare disorder found mostly in young women of oriental origin with a slow spread to other ethnicities.
Although benign and self-limiting in most cases, it can confuse medical professionals with other life-threatening diseases. Early and reliable diagnosis of the disease using imaging technologies and laboratory tests and appropriate management of the symptoms are uppermost to avoid misdiagnosis and mistreatment. The diagnostic flow chart illustrated in the paper should be helpful to medical professionals, particularly to non-specialists including oral health practitioners.
The data used during the study are available from the corresponding author on reasonable request. Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia. Summary Summary.
Symptoms Symptoms. Kikuchi disease is a benign disease of the lymph nodes. The main sign of Kikuchi disease is usually swollen lymph nodes in the neck, which tend to develop suddenly. Showing of 45 View All. Swollen lymph nodes in the neck. Hair loss. Photosensitive skin. Photosensitive skin rashes. Sensitivity to sunlight. Skin photosensitivity. Sun sensitivity. Decreased blood leukocyte number. Low white blood cell count. Mild fever. Mouth ulcer. Fullness of eyelids.
Puffy eyelids. Puffy lids. Swelling of eyelids. Itchy skin. Skin itching. Blistering, generalized. Abnormality in area between air sacs in lung. Low number of red blood cells or hemoglobin. Joint pain. High ESR. High liver enzymes. High lymphocyte count. Muscle ache. Muscle pain. Low blood neutrophil count.
Low neutrophil count. Increased spleen size. Low platelet count. Generalized swelling of lymph nodes. Swollen lymph nodes affecting all regions of the body. Kikuchi disease will usually clear up on its own within one to six months. Sign up for our Health Tip of the Day newsletter, and receive daily tips that will help you live your healthiest life. Chaitanya BN, Sindura C. Kikuchi's disease. J Oral Maxillofac Pathol. Your Privacy Rights. To change or withdraw your consent choices for VerywellHealth.
At any time, you can update your settings through the "EU Privacy" link at the bottom of any page. These choices will be signaled globally to our partners and will not affect browsing data.
We and our partners process data to: Actively scan device characteristics for identification. I Accept Show Purposes. Was this page helpful? Thanks for your feedback! Sign Up. An infectious cause, or at least trigger, to KFD has been postulated. Organisms such as Epstein-Barr virus, cytomegalovirus, varicella-zoster virus, human herpes virus-6, human immunodeficiency virus, Yersinia enterocolitica , and Toxoplasma gondii have been implicated, but no convincing causal relationship has been identified [ 6 ].
Some authors have hypothesised that KFD is a self-limited autoimmune condition triggered by virus-infected transformed lymphocytes [ 12 ]. This theory is based on the histopathological features of the disease, which are similar to those seen in viral infections [ 4 ]. However, serological testing and histological staining for viruses have been consistently unhelpful in supporting this theory.
Although SLE may be more prevalent in KFD patients, a clearly defined relationship between the two conditions has not been identified. Care should be taken in making the diagnosis of concomitant SLE in the patient with KFD as the two diseases share some clinical features lymphadenopathy, rash, pyrexia of unknown origin, and arthralgia. Other less frequently reported symptoms are fatigue, arthralgia, rash, and weight loss.
Affected lymph nodes are typically painless, solid, and mobile [ 14 ]. Although cervical nodes are most often affected, nodes in other regions, including the axilla and groin, can be involved.
Routine laboratory indices are typically unhelpful in establishing a diagnosis of KFD. Reported haematological findings are leukopenia, neutropenia, lymphocytosis, thrombocytopenia, or anaemia [ 3 ]. Notably, our patient displayed none of the above abnormalities. Biochemical abnormalities reported in other cases are elevated inflammatory markers C-reactive protein and erythrocyte sedimentation rate , elevated liver transaminases, increased LDH, antinuclear antibody positivity, and reduced complement 3 values [ 6 ].
Our patient had raised inflammatory markers and LDH only, findings which may be present in many infectious or malignant processes. Imaging, such as computed tomography or magnetic resonance imaging, is unhelpful in distinguishing the lymphadenopathy of KFD from other causes.
These imaging tests are often performed, however, to identify a suitable site for lymph node biopsy or excision. The list of differential diagnoses for our patient, and for KFD in general, is long and includes any potential cause of lymphadenopathy and fever. Infectious causes are likely to be considered first, particularly as in our patient who had recently travelled to India.
We considered tuberculosis, toxoplasmosis, human immunodeficiency virus, Epstein-Barr virus, herpes simplex, dengue fever, and mumps. As discussed above, autoimmune conditions such as SLE can cause a presentation similar to KFD and are therefore high in the differential diagnosis. Finally, malignancy both haematological and solid-organ is undoubtedly the most important diagnosis to exclude in a patient presenting with features of KFD. The high LDH levels and lymphadenopathy in our patient made us highly suspicious of lymphoma, and the excisional lymph node biopsy was carried out to exclude or confirm that diagnosis.
The definitive diagnosis of KFD can be made only through lymph node biopsy and histological examination [ 5 ]. The histopathological features of KFD have been classified into three stages: 1 proliferative stage, with expression of histiocytes, plasmacytoid monocytes, and lymphoid cells containing karyorrhectic nuclear fragments and eosinophilic apoptotic debris; 2 necrotising stage, with a degree of coagulative necrosis; and 3 xanthomatous stage, with foamy histiocytes predominating [ 5 , 16 ].
A histological analysis in disputed this theory slightly, suggesting that the xanthomatous stage is not the resolving stage of KFD but is a histological variant of KFD in its own right [ 17 ]. Since KFD is a self-limited illness, often no treatment is required. Supportive measures, including nonsteroidal anti-inflammatory drugs and antipyretics, can be used for relief of lymph node tenderness, arthralgia, and fever.
Corticosteroids are generally reserved for severe cases, or where supportive measures fail to control symptoms [ 4 ]. Other immunosuppressive agents hydroxychloroquine, cyclosporine, and azathioprine have been used successfully in individual cases. One analysis of cases of KFD reported an overall mortality rate of 2. A female patient died of heart failure, in the context of other haematological autoimmune complications including haemolytic anaemia.
Another patient died of pulmonary haemorrhage and a week pregnant woman died from multiorgan failure after developing KFD-triggered hemophagocytic syndrome. Three patients developed KFD after organ transplantation and died of respiratory failure. These patients were immunosuppressed and there were probably other factors than KFD involved in their illness [ 6 ].
This case describes an unusual and unexpected cause of fever and lymphadenopathy in a returning traveller. We believe that the initial focus on infectious causes was the correct approach in this patient, but the case illustrates that a history of foreign travel can sometimes be coincidental rather than directly implicated in such a presentation.
It should also be remembered that the approach to the febrile traveller should not simply focus on potential exposure to infectious diseases, but also on the ethnicity of the patient, which can make the patient more or less predisposed to certain conditions.
Kikuchi-Fujimoto disease is rare and relatively benign, but its clinical features can easily be mistaken for more sinister diseases.
0コメント