kawasaki disease pictures
Kawasaki disease pictures
What is Kawasaki disease? Kawasaki disease is caused by vasculitis of the small and medium sizes blood vessel. It is an idiopathic disorder affecting young children. Kawasaki disease may consist of self limited acute phase with the symptoms and signs last for the first 10 days and later resolves spontaneously. If untreated, 20%-25% will later develop into coronary artery aneurysm.
Kawasaki disease has a high rate in Pacific Islander and Asian children followed by African American children. 15 per 100 000 children under the age of are hospitalized annually. Kawasaki disease is slightly higher in term of incidence in boy. The median age is 2 years old. 72% of cases involved children less than 3 years old. Kawasaki disease is the major causes of heart disease acquired in children and surpassing the rheumatic fever.
The etiology is unclear. Kawasaki disease is common in winter and springs. There is a low rate of recurrent in small children. The infectious agent has been linked with Kawasaki disease, but the pathogenic agents remain unknown. However, there is evidence that Kawasaki disease is associated with group A streptococci or staphylococcus aureus infection. Other factors such as humidifiers; recent carpets cleaning and house near water source are still unclear factors.
Patient with Kawasaki disease may develop other condition such as frank arthritis, arthralgia, diarrhea, abdominal pain, myocardial dysfunction, pericarditis (early course of the disease ), congestive heart failure and infantile periarteritis nodosa.
Infantile periarteritis nodosa is a condition that present with coronary artery aneurysm that is indistinguishable to Kawasaki disease in term of pathological finding.
However, patient with infantile periarteritis is not presented with other signs of Kawasaki disease.
How to diagnose Kawasaki disease? Patient who suffers from Kawasaki disease may present with fever for more than 5 days and present with four or more of the criteria. The criteria are non vesicular but polymorphous rash, bilateral non exudative injection of the conjunctiva, involvement of the mucosal of the respiratory tract such as strawberry tongue, crusting or fissured erythematous mouth and lip and non exudative injected pharyngitis. Other criteria are erythema and edema of the feet and hands and unilateral (more than 1.5cm diameter) of cervical adenopathy.
Patient who present with fewer than four criteria are known as sufferer of incomplete Kawasaki disease. Incomplete Kawasaki disease is common in children less than 1 year of age. This patient may still develop aneurysm.
Kawasaki disease is divided into three phases such as acute phase, subacute phase, and convalescent phase.
The acute phase will last for 1- 2 weeks from the onset of Kawasaki disease. Patient present with high temperature (fever more than 39 celsius). The patient is highly febrile, toxic in appearance and irritable. Changes in the oral mucosa may exceed quickly and last for 1-2 weeks. Patient mobility is often affected by erythema and edema of the feet which are painful in nature. Rash also will be erupted and common in perianal region.
The subacute phase develops from 2- 8 weeks after the Kawasaki disease onset. Patient may heal without treatment. The improvement process is gradual in nature. The patient palm, perianal region, periungual region, and sole will undergo desquamousation and the fever will improve. However, the patient may suffer from the persistent case of arthralgia and arthritis. The subacute phase may also pose a risk of developing coronary artery aneurysm and myocardial infarction especially on the early stages of subacute phase.
The convalescent phase will last from months to years. During this time period, the lab result, and value will return to normal and the remaining symptoms will be resolved. However, patient may suffer from persistent cardiac dysfunction, persistent aneurysm and myocardial infarction. In some patient, aneurysm may resolves.
Patient may also present with unremitting high fever which last for 1-2 weeks. The rash is not bullous or vesicular, but it is erythematous as well as polymorphous. The rash is maculopapular in nature, and the rash may coalesce and later appear to be petechial in nature. The rash is predominantly on the perineum and the trunk. Other changes include non exudative and bilateral conjunctivitis. Patient may undergo oral changes such as crusting of lips, fissure, erythema, the present of strawberry tongue or oropharyngeal erythema. The extremity of the patient may also undergo changes such as erythema of the soles, palms and induration of the feet and hands. Periungual desquamousation may present in the subacute phase. 2-3 months later after the onset, Beau lines or transverse groove across the fingernail is seen. Unilateral cervical adenopathy may present, but it is less likely to be formed as part of the major criteria. Unilateral cervical adenopathy is fleeting and easily missed.
Other presentation may include aseptic meningitis, pancarditis, vulvitis, meatitis, arthralgia and uveitis. Aseptic meningitis patient may be irritable, ataxic, or shows sign of encephalopathy. Pericarditis patient is characterized by having muffled heart sound, tachycardia, gallop rhythm, signs of congestive heart failure and mitral and aortic insufficiency murmur. Vulvitis and meatitis are associated with sterile pyuria and urethritis. Approximately 1/3 of patient may suffer from arthritis. The arthritis is usually non deforming resolved in less than 1 months, onset on the 2nd and 3rd weeks and involved small and large joints. Anterior uveitis may be revealed on the slit lamp examination.
There are no specific test to diagnose the present of Kawasaki disease. The aim of the test is just to exclude other disorder such as Epstein Barr Virus, enterovirus, roseola, toxic shock syndrome, scarlet fever and adenovirus infection.
The white blood cell count is raised, and the ESR and CRP are elevated while the patient is anemic. Platelets count may be high, low, or normal. However, it will raise rapidly at the second weeks of the illness. Platelets count continues to grow up to 1 to 2 * 10 power of 9 in the subacute phase. Other laboratory findings are sterile pyuria, mild cause of hypoalbuminemia, normal protein and glucose with CSF pleocytosis and mild increase in hepatic transaminase.
During the acute phase, dilated heart is detected on the chest X-ray. Echo cardiography may reveal an effusion and shortening fraction. Aneurysm is detected during the 6 days into the illness that peak at 3 weeks or 4 weeks. ECG may reveal prolonged PR interval, ST changes, flat T waves and reduction in the QRS voltage.
Other differential diagnosis may include group A beta hemolytic streptococci infection and measles. These infections closely resemble Kawasaki disease. Toxic shock syndrome (staphylococcal infection with a release of toxin) may resemble Kawasaki disease. However, renal impairment as a result of toxic shock syndrome is rarely found in Kawasaki disease. Leptospirosis, Rocky mountain spotted fever, enterovirus, roseola, Epstein Barr virus and adenovirus may also mimic Kawasaki disease. Other disorders that may mimic Kawasaki disease may include Steven - Johnson Syndrome. The rash is likely to be crusted and vesicular and exudative conjunctivitis may present. History of drug intake is always associated with Steven Johnson syndrome. Hypersensitivity reaction, acute rheumatic fever and juvenile idiopathic arthritis are the remaining disorders that form the differential diagnosis.
The aim of acute treatment is to reduce the inflammation of the coronary arteries and the development of aneurysm. The present of aneurysm may require further imaging to be taken to reduce the incidence of myocardial infarction and coronary artery thrombosis.
The drug requires is immunoglobulin (IVIG) with a dose of 2g/kg as a 1 time dose. Patient who doesn’t responding to IVIG should be retreated. Side effect of IVIG is congestive heart failure fluid overload and aseptic meningitis. 80- 100 mg/kg of aspirin in divided dose is still the mainstay of therapy. Aspirin is used in conjunction with IVIG. A steroid is added. However the role remains unclear. If the patient is unresponsive to IVIG other therapies may include TNF- alpha antagonist, plasma exchange and cyclophosphamide. Additional anticoagulant and anti platelet therapy are considered in the present of the coronary artery abnormalities.
Death occurs because of coronary disease in 0.3% - 2% of cases. The death is mostly associated with myocarditis and myocardial infarction which may present several years later. The male has a higher mortality than female who suffers from Kawasaki disease as a result of giant cell coronary artery aneurysm. (more than 8 mm) .
The complications of Kawasaki disease are pancarditis and aneurysm. Pericardial effusion may accompany the pancarditis. Pancarditis will present on the first 10 days of illness. Aneurysm may occur during the 12- 28 days. The aneurysm may undergo thrombosis and lead to myocardial infarction and death.
The white blood count, ESR and platelet counts of the patient should be monitored weekly until the value return to normal. At 2 weeks and, 6-8 weeks ECG is performed to monitor and rule out the present of aneurysm. The present of aneurysm may require further coronary artery catheterization and imaging studies to be performed especiallly at the 8 to 12 weeks after illness. The present of any cardiac symptoms such as dyspnea on exertion, chest pain and fatigue may require further evaluation of the myocardial function to be taken.
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