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Kikuchi-Fujimoto Disease

 Kikuchi-Fujimoto Disease

  



OVERVIEW

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenopathy, is a rare, benign (noncancerous, nonmalignant) disorder of the lymph nodes, predominantly of young adults and school aged children. While slightly more common in females, it is also seen in males. This disorder presents with similar signs and symptoms as lymphoma, including enlarged lymph nodes (lymphadenopathy) The exact cause is not known. KFD shows up as painful swelling of lymph nodes, often in the neck, along with general symptoms like fever and fatigue.

KFD is similar to a type of inflammatory response called granulomatous response. When the lymph nodes are examined under a microscope, specific changes may be seen such as tissue necrosis (cell death) and an absence of a particular type of immune cell called neutrophils. Instead, the necrotic areas mainly contain remnants of cells undergoing apoptosis, where the cell nucleus breaks down. In simpler terms, KFD is a unique condition where the affected tissue shows signs of cell death, but the immune cells generally associated with that kind of reaction are missing. This unusual pattern makes it distinct from other diseases with similar symptoms.

Usually, this disease does not cause severe problems and gets better on its own. However, in some people, it can be associated with autoimmune disorders, conditions where the immune system mistakenly attacks the body’s cells and tissues.

Symptoms

KFD can cause a person to experience swollen lymph nodes. This swelling is generally unilateral, which means it only affects one side of the body. Evidence notes that KFD affects more than one area of lymph nodes in 22% of people.

Fever is the most common symptom of KFD. Other symptoms of KFD can include:

rashes

joint pain

fatigue

sore throat

upper respiratory problems

swelling of the liver and spleen

weight loss

night sweats

cough

headache

low white blood cell count

nausea

vomiting

diarrhea

Temporary skin issues can also occur alongside KFD. Evidence states that up to 40% of people with KFD also have a skin issue, such as:

a measles-like rash

rash on the face

spots or lesions on the face

hard bumps on the back and arms

scaling of the skin

itching

hair loss

ulcers on the back of the throat

Symptoms of KFD typically develop over 2–3 weeks. These symptoms generally clear up over the next 4 months.

Causes

What causes KFD is currently unknown. Researchers believe that it may occur due to an immune response to a virus or autoimmune trigger.

Research from 2021 notes that KFD diagnoses have been associated with the following conditions:

Epstein-Barr virus (EBV)

herpes simplex virus (HSV)

varicella-zoster virus (VZV)

autoimmune conditions such as systemic lupus erythematosus (SLE) and Sjogren’s syndrome

Diagnosis

There are various Trusted Source health conditions that can have similar symptoms to KFD. These include:

lymphoma, a form of cancer

tuberculosis (TB), a bacterial infection that affects the lungs

adult-onset Still’s disease (AOSD), a rare form of inflammatory arthritis

systemic lupus erythematosus (SLE), an autoimmune condition

polymyositis, an inflammatory disease that affects muscles

scleroderma, an autoimmune disease that causes skin and connective tissue to harden

thyroiditis, which is an inflammation of the thyroid glands

It is important that a doctor correctly diagnoses a person so that they can receive the right treatment. Research from 2016 notes that 58% of people with KFD within a specific study were misdiagnosed as having TB.

When diagnosing KFD, a doctor may perform a physical exam. They can feel a person’s lymph nodes to see if they are swollen.

A doctor may also order an imaging test, such as an ultrasound or magnetic resonance imaging (MRI) to check a person for enlarged lymph nodes.

A doctor confirms a KFD diagnosis by taking a sample from a person’s lymph node. This is known as a biopsy. The doctor then sends the biopsy sample to a laboratory for testing. If a person has KFD, their sample will show signs of cell death. Additionally, there will be a lack of granulocytes, a type of white blood cell.

There is no specific KFD blood test. However, a doctor can take a blood sample from a person to test their erythrocyte sedimentation rate (ESR). ESR tests how long it takes for a person’s red blood cells to settle on the bottom of a test tube.

A person’s red blood cells should settle slowly on the bottom of a test tube. However, if inflammation is present, their red blood cells will stick together. These clumps of cells will sink more quickly to the bottom of the test tube.

Treatment 

KFD is a self-limiting condition. This means that it usually goes away by itself without treatment.

A person can take pain medication, such as acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs) to help manage KFD-related pain. A doctor may treat a person who has severe symptoms or a reoccurrence of KFD. The KFD reoccurrence rate in adults is 3–4%.

Treatment options may include:

oral corticosteroids, a form of anti-inflammatory medication

intravenous immunoglobulin (IVIg), which provides a person with antibodies via a vein

hydroxychloroquine, a medication used for inflammation

Type of Doctor Department :Pediatrics

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