Definition of Mucocutaneous lymph node syndrome (Kawasaki disease)
Kawasaki disease is a condition that causes inflammation in the walls of medium-sized arteries throughout the body, including the coronary arteries, which supply blood to the heart muscle. Kawasaki disease is also called mucocutaneous lymph node syndrome because it also affects lymph nodes, skin, and the mucous membranes inside the mouth, nose and throat.
Signs of Kawasaki disease, such as a high fever and peeling skin, can be frightening. The good news is that Kawasaki disease is usually treatable, and most children recover from Kawasaki disease without serious problems.
Symptoms of Mucocutaneous lymph node syndrome (Kawasaki disease)
Kawasaki disease symptoms appear in phases.
Signs and symptoms of the first phase may include:
- Fever which often is higher than 102.2 F (39 C) and lasts more than five days
- Extremely red eyes (conjunctivitis) without a thick discharge
- A rash on the main part of the body (trunk) and in the genital area
- Red, dry, cracked lips and an extremely red, swollen tongue (strawberry tongue)
- Swollen, red skin on the palms of the hands and the soles of the feet
- Swollen lymph nodes in the neck and perhaps elsewhere
In the second phase of the disease, your child may develop:
- Peeling of the skin on the hands and feet, especially the tips of the fingers and toes, often in large sheets
- Joint pain
- Abdominal pain
In the third phase of the disease, signs and symptoms slowly go away unless complications develop. It may be as long as eight weeks before energy levels seem normal again.
When to see a doctor
If your child has a fever that lasts more than four days, contact your child’s doctor. Or see your child’s doctor if your child has a fever along with four or more of the following signs and symptoms:
- Redness in both eyes
- A very red, swollen tongue
- Redness of the palms or soles
- Skin peeling
- A rash
- Swollen lymph nodes
Treating Kawasaki disease within 10 days of its onset may greatly reduce the chances of lasting damage.
No one knows what causes Kawasaki disease, but scientists don’t believe the disease is contagious from person to person. A number of theories link the disease to bacteria, viruses or other environmental factors, but none has been proved. Certain genes may increase your child’s susceptibility to Kawasaki disease.
Three things are known to increase your child’s risk of developing Kawasaki disease, including:
- Age. Children under 5 years old are most at risk of Kawasaki disease.
- Sex. Boys are slightly more likely than girls are to develop Kawasaki disease.
- Ethnicity. Children of Asian descent, such as Japanese or Korean, have higher rates of Kawasaki disease.
Complications of Mucocutaneous lymph node syndrome (Kawasaki disease)
Kawasaki disease is a leading cause of acquired heart disease in children, but with effective treatment, only a small percentage of children have lasting damage.
Heart complications include:
- Inflammation of blood vessels (vasculitis), usually the coronary arteries, that supply blood to the heart
- Inflammation of the heart muscle (myocarditis)
- Heart valve problems (mitral regurgitation)
- Abnormal heart rhythm (dysrhythmia)
Any of these complications can damage your child’s heart. Inflammation of the coronary arteries can lead to weakening and bulging of the artery wall (aneurysm). Aneurysms increase the risk of blood clots forming and blocking the artery, which could lead to a heart attack or cause life-threatening internal bleeding.
For a very small percentage of children who develop coronary artery problems, Kawasaki disease is fatal, even with treatment.
Preparing for your appointment
You’ll probably first see your family doctor or pediatrician. However, in some cases your child may also be referred to a doctor who specializes in treating children’s hearts (pediatric cardiologist).
Because appointments can be brief and there’s often a lot of ground to cover, it’s a good idea to be well prepared. Here’s some information to help you get ready for your appointment, as well as what you can expect from your child’s doctor.
What you can do
- Write down any symptoms your child is experiencing, including any that may seem unrelated. Try to keep track of how high your child’s fever has been and how long it has lasted.
- Make a list of any medications, vitamins or supplements that your child is taking.
- Ask a family member or friend to join you, if possible. Sometimes it can be difficult to soak up all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
- Write down questions to ask your doctor.
Your time with your doctor is limited, so preparing a list of questions will help you make the most of your appointment. List your questions from most important to least important in case time runs out. For Kawasaki disease, some basic questions to ask your child’s doctor include:
- What’s the most likely cause of my child’s symptoms?
- Are there any other possible causes for his or her symptoms?
- Does my child need any tests?
- How long will the symptoms last?
- What treatments are available, and which do you recommend?
- What are the possible side effects of the treatments?
- Are there any steps I can take to make my child more comfortable?
- What signs or symptoms should I watch for that might indicate that he or she is getting worse?
- What’s my child’s long-term prognosis?
- Are there any brochures or other printed material that I can take home with me? What websites do you recommend visiting?
In addition to the questions that you’ve prepared to ask your doctor, don’t hesitate to ask any additional questions that may come up during your appointment.
What to expect from your doctor
Your child’s doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your child’s doctor may ask:
- When did your child begin experiencing symptoms?
- How severe are the symptoms? How high has your child’s fever been? How long did it last?
- What, if anything, seems to improve the symptoms?
- What, if anything, appears to worsen the symptoms?
- Has your child been exposed to any infectious diseases?
- Has your child been taking any medications?
- Does your child have any allergies?
What you can do in the meantime
Initially, you can give your child acetaminophen (Tylenol, others) or ibuprofen (Advil, Children’s Motrin, others) to reduce the fever and make him or her more comfortable. However, these products are not recommended for low fevers, and treating your child’s fever will make it difficult to accurately measure how severe the fever is and the actual duration of the fever. Additionally, don’t give your child aspirin without consulting the doctor first. In children and teenagers, taking aspirin during certain viral infections, such as chickenpox, has been associated with the development of Reye’s syndrome — a rare but serious disorder. Kawasaki treatment is a rare exception to the rule against aspirin use in children but only when given under the supervision of your child’s doctor.
Tests and diagnosis
There’s no specific test available to diagnose Kawasaki disease. Diagnosis largely is a process of ruling out diseases that cause similar signs and symptoms, including:
- Scarlet fever, which is caused by streptococcal bacteria and results in fever, rash, chills and sore throat
- Juvenile rheumatoid arthritis
- Stevens-Johnson syndrome, a disorder of the mucous membranes
- Toxic shock syndrome
- Certain tick-borne illnesses, such as Rocky Mountain spotted fever
The doctor may do a physical examination and have your child take other tests to help in the diagnosis. These tests may include:
- Urine tests. These tests help rule out other diseases.
- Blood tests. Besides helping to rule out other diseases, blood tests look at white blood cell count, which is likely to be elevated, and the presence of anemia and inflammation, indications of Kawasaki disease.
- Electrocardiogram. This test uses electrodes attached to the skin to measure the electrical impulses of your child’s heartbeat. Kawasaki disease can cause heart rhythm complications.
- Echocardiogram. This test uses ultrasound images to show how well the heart is functioning and provides indirect evidence on how the coronary arteries are functioning.
Treatments and drugs
To reduce the risk of complications, your child’s doctor will want to begin treatment for Kawasaki disease as soon as possible after the appearance of signs and symptoms, preferably while your child still has a fever. The goals of initial treatment are to lower fever and inflammation and prevent heart damage.
To accomplish those goals, your child’s doctor may recommend:
- Gamma globulin. Infusion of an immune protein (gamma globulin) through a vein (intravenously) can lower the risk of coronary artery problems.
- Aspirin. High doses of aspirin may help treat inflammation. Aspirin can also decrease pain and joint inflammation, as well as reduce the fever. Kawasaki treatment is a rare exception to the rule against aspirin use in children.
Because of the risk of serious complications, initial treatment for Kawasaki disease usually is given in a hospital.
After the initial treatment
Once the fever goes down, your child may need to take low-dose aspirin for at least six weeks and longer if he or she develops a coronary artery aneurysm. Aspirin helps prevent clotting.
However, if your child develops flu or chickenpox during treatment, he or she may need to stop taking aspirin. Taking aspirin has been linked to Reye’s syndrome, a rare but serious illness that can affect the blood, liver, and brain of children and teenagers after a viral infection.
Without treatment, Kawasaki disease lasts an average of 12 days, though heart complications may be evident later and be longer lasting. With treatment, your child may start to improve soon after the first gamma globulin treatment.
Monitoring heart problems
If your child has any indication of heart problems, the doctor may recommend follow-up tests to monitor heart health at regular intervals, often at six to eight weeks after the illness began. If your child develops continuing heart problems, the doctor may refer you to a doctor who specializes in treating heart disease in children (pediatric cardiologist). In some cases, a child with a coronary artery aneurysm may require:
- Anticoagulant drugs. These medications — such as aspirin, clopidogrel (Plavix), warfarin (Coumadin) and heparin — help prevent clots from forming.
- Coronary artery angioplasty. This procedure opens arteries that have narrowed to the point that they impede blood flow to the heart.
- Stent placement. This procedure involves implanting a device in the clogged artery to help prop it open and decrease the chance of reblockage. Stent placement may accompany angioplasty.
- Coronary artery bypass graft. This operation involves rerouting the blood around a diseased coronary artery by grafting a section of blood vessel from the leg, chest or arm to use as the alternate route.
Coping and support
Find out all you can about Kawasaki disease so that you can make informed choices with your child’s health care team about treatment options. Keep in mind that most children with Kawasaki disease recover completely, though it may be a little while before your child is back to normal and not feeling so tired and irritable. The Kawasaki Disease Foundation offers trained support volunteers to families currently dealing with the disease.