Mucocutaneous lymph node syndrome

Also known as Kawasaki disease, it is a rare condition characterized by the inflammation in the walls of the small and medium-sized blood vessels, particularly the coronary arteries in the body. This syndrome primarily affects children under the age of five that present high temperature, rash, cracked dry lips and swollen glands in the neck. This disease usually resolves on its own within 4-8 weeks.

Causes

The exact cause of this disease is not known. Research indicates that the disease can be linked to microbes and various environmental factors. Certain genes are also attributed to increasing the susceptibility to developing this condition. One theory suggests that this syndrome may be an autoimmune condition when the affected individual’s immune system accidentally attacks the healthy tissues of the body. Some of the risk factors that can increase the child’s risk of developing Kawasaki disease includes children under the age of 5, boys are more likely to develop this condition and belonging to certain ethnic background such as being Asian or Pacific descent.

Symptoms

This condition usually occurs in three phases. The symptoms presenting in the first phase includes high fever lasting for more than three days, inflammation of the eyes, rash, dry cracked lips, swollen lymph nodes in the neck or elsewhere and irritability. During the second phase, the affected child may develop peeling of the skin on the hands and the feet, vomiting, joint pain, diarrhea and abdominal pain. The symptoms go away during the third phase of the disease unless complications can occur. It can take as long as eight weeks for the child to become completely normal. This disease does not appear to be transmitted from one person to another. Although several people can be exposed to this disease, only a few of them continue to develop the associated symptoms.

Diagnosis

No single test can diagnose this condition. The diagnosis is possible with the identification of features associated with this disease, a physical examination and with various tests. Various diagnostic tests may also be required to rule out other conditions presenting similar symptoms such as measles, toxic shock syndrome, scarlet fever and juvenile rheumatoid arthritis. Additional tests to help with the diagnosis include urine tests and the blood tests which can present an elevated level of white blood count and the presence of anemia. An electrocardiogram measures the electrical impulses of the heartbeat that may be used to determine complications of the disease and the echocardiogram to identify coronary artery abnormalities.

Treatment

Treatment should be initiated soon after the diagnosis as this disease can present serious complications. The treatment includes medications to ease the pain, reduce fever and to help with the inflammation. An intravenous immunoglobulin is a solution of antibodies that is administered directly into the vein which can help improve the symptoms within 36 hours.

References

http://www.mayoclinic.org/diseases-conditions/kawasaki-disease/diagnosis-treatment/treatment/txc-20259803

http://www.nhs.uk/Conditions/Kawasaki-disease/Pages/Treatment.aspx

http://www.dermnetnz.org/topics/kawasaki-disease/