Skip to main content

Kikuchi Disease

 Kikuchi Disease

Disease Overview

Kikuchi’s disease, also known as histiocytic necrotizing lymphadenopathy, is a rare, benign, (noncancerous, nonmalignant) disorder of the lymph nodes of young adults, predominantly of young women. This disorder is often mistaken for malignant lymphoma, especially cervical adenopathy because the symptoms are very similar. The lesions, or tissue anomalies, of this disorder cause the lymph nodes to become enlarged (lymphadenopathy), inflamed and painful. The exact cause of Kikuchi’s disease is not known. Perhaps the primary threat is a misdiagnosis of a malignant lymphoma.

Signs & Symptoms

Kikuchi’s disease is a rare nonmalignant disorder that affects the lymph nodes. Lymph nodes are present throughout the body as small oval structures that filter lymph fluid, fight infection and form white blood cells and blood plasma cells. In addition to swelling and pain in the abdomen the lymph nodes on the side of the neck and near the salivary glands are often be affected.

Affected individuals may develop mild fever, night sweats, muscle pain (myalgia) and a rash. Less common symptoms include headaches, fatigue, joint pain (arthralgia), and nausea and vomiting. In some cases, affected individuals may experience abnormal enlargement of the liver or spleen (hepatosplenomegaly). The symptoms of Kikuchi’s disease may develop slowly over two to three weeks.

For reasons that are unclear the abnormal tissue growth and inflammation in Kikuchi’s disease usually clears up, spontaneously, within a few weeks or months without further therapy (self-limited disease).

Causes

The exact cause of Kikuchi’s disease is not known (idiopathic). Many researchers suspect that a virus may cause this disorder. Others, however, believe that the disorder, like systemic lupus erythematosus, is a self-limited autoimmune condition. Autoimmune disorders are caused when the body’s natural defenses against “foreign” or invading organisms begin to attack healthy tissue for unknown reasons.

Disorders with Similar Symptoms

Symptoms of the following disorders can be similar to those of Kikuchi’s disease. Comparisons may be useful for a differential diagnosis:

Burkitt’s lymphoma is a cancer of the lymphatic system that affects the lymph nodes as well as other areas of the body. Tumors may occur in the kidneys, sex glands, jaw, bone marrow, or central nervous system as well as the lymph nodes. Burkitt’s lymphoma may be infectious. This disorder occurs often in children living in Central Africa, and is associated with the Epstein-Barr virus.

Hodgkin’s disease is a form of lymphatic cancer featuring tumorous lymph nodes. Fever, night sweats and weight loss may occur along with swollen lymph nodes. The diagnosis of Hodgkin’s disease is confirmed if expert pathological examination shows the presence of so-called Reed-Sternberg cells. The exact cause of Hodgkin’s disease is not known. (For more information on this disorder choose “Hodgkin” as your search term in the Rare Disease Database.)

Malignant lymphoma describes a malignant tumor of the lymphoid tissue that is cancerous. Within the lymph system a type of white blood cell (lymphocyte) circulates. Although the structure of these white blood cells may vary with the type of lymphoma present, the effects are usually similar. The appearance of large lymph nodes in the neck is usually followed by fever, weakness, weight loss and anemia. When there is widespread involvement of the lymphoid tissue, the spleen and liver may also enlarge.

The following conditions have been associated with Kikuchi disease in some patients. They are not necessary for a differential diagnosis:

Pancytopenia – a reduction in the number of red and white blood cells and platelets in the circulating blood.

Splenomegaly – an abnormal enlargement of the spleen.

Still’s Disease – also called juvenile rheumatoid arthritis or rheumatic arthritis, is a form of arthritis that usually, but not exclusively, affects the larger joints of children. In addition to high intermittent fever and rash, patients present with inflammation of several thin sheets of tissue that line certain areas of the body (serous membranes). Consequently, the lymph nodes (lymphadenopathy) are affected. (For more information on this disorder, please choose “Rheumatic Arthritis” as your search term in the Rare Disease Database.)

Standard Therapies

Treatment of Kikuchi's disease is symptomatic and supportive. Usually the disorder resolves itself spontaneously within a few weeks or months. Analgestics-antipyretics and nonsteroidal anti-inflammatory (NSAIDs) may be used to treat pain, tenderness and fever associated with lymphadenopathy. In extremely rare cases, Kikuchi's disease may recur.

Kikuchi's disease is diagnosed by removing tissue with a fine needle that is attached to a syringe. The tissue is then examined under a microscope to determine whether the patient has the disease.

TYPE OF DOCTOR AND DEPARTMENT: Pathology SPECIALIST CAN DIAGNOSE THIS DISEASE. 

Comments

Post a Comment

Popular posts from this blog

Charge Syndrome

Overview CHARGE syndrome is a recognizable genetic syndrome with known pattern of features. It is an extremely complex syndrome, involving extensive medical and physical difficulties that differ from child to child. CHARGE syndrome is correlated with genetic mutation to CHD7 and the prevalence of CHARGE syndrome is 1:10,000-1:15,000 live births. Babies with CHARGE syndrome are often born with life-threatening birth defects. They spend many months in the hospital and undergo many surgeries and other treatments. Swallowing and breathing problems make life difficult even when they come home. Most have hearing two little girls sitting on a carpet, one girl has a trach and is biting her finger.loss, vision loss, and balance problems that delay their development and communication. Despite these seemingly insurmountable obstacles, children with CHARGE syndrome often far surpass their medical, physical, educational, and social expectations. One of the hidden features of CHARGE syndrome is the ...
7 comments
Read more

HMSN Type I

 HMSN Type I Overview Hereditary motor and sensory neuropathy type 1 (HMSN I), also known as Charcot-Marie-Tooth disease type 1 (CMT1), is a group of inherited neurological disorders affecting the peripheral nerves. It is characterized by progressive muscle weakness and wasting, primarily in the feet and legs, as well as sensory loss. HMSN I is the most common form of HMSN and is typically caused by a duplication on chromosome 17p11.2-p12, including the PMP22 gene.  Symptoms Numbness and tingling: Often starting in the feet and potentially extending to the hands, these sensations can mimic poor circulation.  Reduced ability to feel pain and temperature: This can lead to unnoticed injuries and complications.  Motor Symptoms: Muscle weakness and wasting: Primarily affecting the distal muscles (those farthest from the body's core), such as the feet and hands.  Foot deformities: High arches (pes cavus) and hammer toes are frequently observed.  Gait abnormalit...
Post a Comment
Read more

Sjogren's syndrome

Sjogren's syndrome Overview Sjogren's (SHOW-grins) syndrome is a disorder of your immune system identified by its two most common symptoms — dry eyes and a dry mouth. The condition often accompanies other immune system disorders, such as rheumatoid arthritis and lupus. In Sjogren's syndrome, the mucous membranes and moisture-secreting glands of your eyes and mouth are usually affected first — resulting in decreased tears and saliva. Although you can develop Sjogren's syndrome at any age, most people are older than 40 at the time of diagnosis. The condition is much more common in women. Treatment focuses on relieving symptoms. Symptoms The two main symptoms of Sjogren's syndrome are: Dry eyes . Your eyes might burn, itch or feel gritty — as if there's sand in them. Dry mouth. Your mouth might feel like it's full of cotton, making it difficult to swallow or speak. Some people with Sjogren's syndrome also have one or more of the following: Joint pain, swel...
Post a Comment
Read more