Table of Contents
Introduction
The disease was described by Kikuchi and Fujimoto in Asia. Kikuchi-Fujimoto was first seen in Japanese patients and mostly occurs among Asians. The Kikuchi-Fujimoto disease (KFD) is a rare disease. It is characterized by inflammation of the lymph nodes. Asian young adults are most commonly affected but it is seen worldwide. KFD has been reported in people of various ethnic backgrounds all over the world. It typically affects young adults (those under the age of 40), but it can affect people of any age. Most reports show female predominance, but some Asian studies suggest that the male-to-female ratio is closer to 1:1. The cause of Kikuchi-Fujimoto is unknown. The diagnosis of this disease is difficult. It may be misdiagnosed as benign lymphadenopathies (swelling of the lymph nodes) or infectious lymphadenitis (inflammation of the lymph nodes).
What Is Kikuchi- Fujimoto Disease?
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign (non-cancerous) condition that affects the lymph nodes. It is characterized by inflammation of the lymph nodes, which can cause enlargement and tenderness. KFD most commonly affects young adults, with a higher incidence in females, and is more prevalent in Asia, particularly in Japan. The cause of KFD is unknown, but it is thought to be related to an abnormal immune response. It may also be associated with viral infections, such as Epstein-Barr virus or human herpesvirus. The disease usually presents with symptoms such as fever, night sweats, and fatigue. Enlarged lymph nodes, often in the neck area, are also common symptoms. A prompt diagnosis is required for proper medical intervention.
What Causes Kikuchi- Fujimoto Disease?
The cause of KFD remains unclear. The two most common causes of KFD are infectious and auto-immune diseases. The disease does not respond to antibiotics.
The following are the causes of the KFD:
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Virus - Human herpesviruses 6, 7, and 8; varicella-zoster; rubella; cytomegalovirus; hepatitis B virus; human immunodeficiency virus; human T-lymphotropic virus type 1; and dengue virus. Epstein-Barr virus, herpes simplex virus, varicella-zoster virus, human herpesviruses 6, 7, and 8; parvovirus B19; paramyxovirus; parainfluenza are responsible for causing KFD.
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Other Agents - Brucella, Bartonella henselae, Yersinia enterocolitica, Toxoplasma gondii, Entamoeba histolytica, and Mycobacterium szulgai also causes KFD.
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Genetic Factor - Human leukocyte antigen (HLA) caused KFD. These alleles are more common in Asians and highly rare or nonexistent in whites, which may explain why this condition affects Asians more frequently than whites.
What Are the Risk Factors for Kikuchi- Fujimoto Disease?
The following are the risk factors of KFD:
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Meningitis (inflammation of the brain).
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Status epilepticus (a condition in which patients suffer from seizures).
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Interstitial lung diseases.
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Myocarditis (inflammation of the layer of the heart).
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Acute renal failure (kidney failure).
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Hemophagocytic syndrome (a hematological condition that occurs in children and adults).
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Sickle cell anemia (the condition in which red blood cells become sickled cells).
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Breast, stomach, and oral cancer.
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Patients with pacemakers (pacemakers are artificial devices placed inside the heart to control heartbeat).
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Auto-immune diseases (the condition in which the body starts attacking its own body).
What Are the Symptoms of Kikuchi- Fujimoto Disease?
The Kikuchi-Fujimoto disease is an acute to subacute course self-limiting disease that progresses over several weeks. Most of the cases of KFD involve posterior cervical lymph nodes (60 to 90 %) with the involvement of axillary lymph nodes. Cervical lymph nodes refer to lymph nodes present in the neck region. Rarely, the disease manifests in extranodal sites, most usually in the skin, but also rarely in the bone marrow and liver. The face and upper body are most commonly affected by cutaneous involvement.
The following are the symptoms of the KFD:
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Mild fever.
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Lymphadenopathy (swelling of the lymph).
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The lymph node is tender and painful.
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Weight loss.
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Nausea and vomiting.
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Weakness.
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Arthralgia (joint pain).
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Night sweats.
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Upper respiratory symptoms.
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Sore throat.
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Hepatomegaly (swelling of the liver).
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Splenomegaly (swelling of the spleen).
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Rashes.
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Nodules in the skin.
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Papule (small bump-like formation in the skin).
Signs:
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Anemia (low amount of hemoglobin in the blood).
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Rise in the erythrocyte level (red blood cell level).
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Leukopenia (low white blood cell count).
How to Diagnose Kikuchi- Fujimoto Disease?
The diagnosis of Kikuchi-Fujimoto disease (KFD) is typically based on a combination of clinical presentation, laboratory tests, and a biopsy of the affected lymph node. It is important to note that KFD can sometimes be misdiagnosed as other conditions such as tuberculosis, lymphoma, or systemic lupus erythematosus (SLE) because of the similarity of the symptoms. Therefore, a thorough evaluation and proper diagnostic tests are essential to ensure an accurate diagnosis.
Here are some of the diagnostic methods used to identify KFD:
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Clinical Presentation: Patients with KFD often present with fever, night sweats, fatigue, and enlarged lymph nodes, typically in the neck area. A thorough physical examination can provide valuable information for diagnosis.
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Laboratory Tests: Blood tests are typically performed to help rule out other conditions that may present similarly to KFD. These tests may include a complete blood count (CBC), erythrocyte sedimentation rate (ESR), and liver function tests (LFTs). Patients with KFD may have mild anemia and elevated ESR.
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Biopsy: A biopsy of the affected lymph node is usually required for a definitive diagnosis of KFD. The biopsy typically shows characteristic changes, including necrosis (cell death) and an increased number of immune cells called histiocytes and lymphocytes. The biopsy can be performed through a fine-needle aspiration or an excisional biopsy.
How to Treat Kikuchi- Fujimoto Disease?
The treatment for KFD is usually supportive, with the goal of relieving symptoms. The Kikuchi-Fujimoto disease is self-limiting and resolves itself in a few months. Severe fatal cases have been documented, and the recurrence rate is only between three and four percent. Since there is no specific therapy for these patients, the goal of treatment is to reduce symptoms (with rest, analgesics, and antipyretics). Corticosteroid therapy may be beneficial for patients with relapsing disease or a more severe clinical course. Nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to reduce fever and pain. In severe cases, corticosteroids or immunosuppressive drugs may be prescribed to reduce inflammation.
Conclusion:
The Kikuchi-Fujimoto disease presents considerable diagnostic difficulties. To prevent a false lymphoma diagnosis that would require severe treatment and excessive testing, pathologists should be knowledgeable about this condition. The best specimen for diagnosing KFD is an excisional lymph node biopsy, although in situations when there is insufficient material, immunohistochemical stains may be beneficial. Clinically, SLE should be checked out in all patients with KFD, as it is one of the most significant differential diagnoses.
