Mucocutaneous lymph node syndrome, also known as Kawasaki disease, was first identified in 1942 by Japanese physician Tomisaku Kawasaki. This disease primarily affects infants and young children, with clinical features including fever, rash, conjunctivitis, indurative erythema of the hands and feet, and cervical lymphadenopathy. It is currently considered to be a distinct infectious or allergic disorder.

bubble_chart Epidemiology

This disease was first reported in Japan, and there have been tens of thousands of cases to date. It has also been observed in other European and American countries. In China, hundreds of cases have been reported since 1975, but the actual number of cases is higher than the reported figures. (1) Regional distribution: Found worldwide, with a higher prevalence in Japan. (2) Ethnicity: More common in Asian populations; in the U.S., Asians have a higher incidence than Black and White populations. (3) Age: Predominantly affects children aged 2 months to 4 years, with a peak at 2 years old; rare in adults. (4) Gender: More common in boys, with a male-to-female ratio of 1.5–1.8:1. (5) Contagiousness: Among 520 cases observed in Japan, no human-to-human transmission was found. (6) Season: Occurs year-round, with higher incidence from July to September in China and Japan, and from February to May in the U.S. (7) Living conditions: More common among middle- to upper-class residents, often following prodromal symptoms of upper respiratory infections.

Pathology

This disease is a systemic vasculitis, primarily involving multiple small artery inflammations, characterized by infiltration of monocytes, lymphocytes, and plasma cells, with rare presence of multinucleated cells. Later stages involve proliferative changes and scar formation. It can affect multiple organs, including the heart, lungs, liver, brain, kidneys, intestines, and joints. However, the disease is self-limiting. Complications such as coronary arteritis, periarteritis, coronary artery thrombosis, or rupture of coronary artery aneurysms can be fatal. Papillary muscle dysfunction may lead to mitral regurgitation or mitral valve prolapse.

The cause of the disease

The cause of the disease is largely unknown. Currently, it is believed that (1) it may be caused by bacteria, viruses, or rickettsiae, but opinions vary and no consensus has been reached. (2) Certain disease-causing factors may trigger type III hypersensitivity reactions, and electron microscopy reveals antigen-antibody complexes in the vascular walls, suggesting a possible link to autoimmunity. (3) Some propose an association with certain chemicals (e.g., detergents, mercury).

bubble_chart Clinical Manifestations

The clinical course of this disease is roughly divided into the following stages: 1. **Acute stage [third stage]**: Within two weeks of onset, the main clinical manifestations predominate during this period. 2. **Subacute stage**: 15–25 days or longer after the illness. Symptoms primarily involve cardiac involvement and arthritis. 3. **Stage of convalescence**: Two months after onset, various symptoms gradually disappear, but symptoms due to coronary vascular damage may persist.
**(1) Major Manifestations**

1. **Fever**: Persistent unexplained irregular or remittent fever lasting more than 5 days, which may exceed 40°C. Antibiotics are ineffective, and the febrile course can last 1–3 weeks, averaging about 11 days.
2. **Conjunctival congestion**: Appears 3–4 days after illness onset and resolves when the fever subsides. No purulent discharge, no ciliary congestion, and no damage to the cornea or inner eye.
3. **Changes in lips and oral mucosa**: Lips are red, cracked, and may bleed. Diffuse congestion of the oral and pharyngeal mucosa, with congested and prominent lingual papillae, resembling a "strawberry tongue."
4. **Rash**: Polymorphous erythema or scarlatiniform rash over the body, without vesicles, crusting, or pigmentation.
5. **Changes in extremities**: Hard, red, and edematous skin on the hands, feet, palms, toes, and fingertips, with fusiform swelling of small joints, accompanied by pain and functional impairment. During resolution, membranous desquamation occurs at the junction of the nail bed and skin on fingers and toes.
6. **Lymphadenopathy**: Enlarged, firm, and tender lymph nodes in the neck, postauricular, and occipital regions, presenting as transient non-suppurative changes.
7. **Facial nerve palsy**: Although its incidence is not high, it holds significant diagnostic value.

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1. **Cardiovascular system changes**: Occurrence rate of 4–20%, more common in males than females. While diagnostically considered minor, these changes are severe for the patient. The disease has a 1–2% mortality rate, all due to cardiovascular involvement. Manifestations include myocarditis, pericarditis, heart failure, myocardial infarction, cardiac arrest, and arrhythmias.
2. **Digestive system changes**: Vomiting, abdominal pain, diarrhea, mucous stools, and occasionally jaundice, hepatomegaly, and elevated GPT.
3. **Urinary changes**: Proteinuria and increased white blood cells, mostly self-limiting.
4. **Neurological changes**: May include aseptic meningitis, increased intracranial pressure, seizures, and occasionally limb paralysis.
5. **Blood tests**: Increased total white blood cell count, neutrophilia with a left shift, elevated platelets, accelerated ESR, and grade I anemia. CRP (+), normal ASO, and increased α2-globulin.

bubble_chart Diagnosis

It is mainly manifested in the first six items, and the diagnosis can be made if four or more manifestations, including fever, are present. There is no specific diagnostic method, and secondary manifestations serve as references.

bubble_chart Treatment Measures

There is currently no specific treatment for

this disease. Supportive therapy and symptomatic treatment are adopted to reduce vasculitis and prevent or minimize cardiac damage.

1. Supportive therapy involves maintaining adequate hydration, calories, and vitamins.
2. Aspirin is used for its antipyretic and antiplatelet aggregation effects to prevent thrombosis. Dosage: 50–100 mg/kg/day. After fever subsides, 30 mg/kg/day can be used for 2 months. Combined with oral dipyridamole at 3 mg/kg/day, it enhances the antiplatelet aggregation effect of aspirin. Other platelet function inhibitors include flurbiprofen at 4 mg/kg/day, reduced to 2 mg/kg/day after fever subsides.
3. Corticosteroids reduce fever, improve skin and mucous membrane symptoms, and have nonspecific anti-inflammatory effects, which may shorten the disease course. However, they may promote coronary artery aneurysm formation, making their use controversial. They should not be routinely administered. The usual prednisone dosage is 1–2 mg/kg/day or high-dose short-term therapy.
4. Intravenous human gamma globulin at 400 mg/kg/day for 5 days can reduce cardiovascular complications. Additionally, it lowers the risk of bacterial and viral infections, and plasma transfusion is also effective.
5. Currently, no antibiotics are effective, but they may still be used clinically during the acute phase to prevent secondary infections.

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bubble_chart Prognosis

The disease is self-limiting, with most cases resolving on their own, and about 3% may relapse once or twice. The fatality rate is 1-2%. Some patients were followed up for six months to eight years, and a very small number still developed coronary artery aneurysms and large artery inflammation.

bubble_chart Differentiation

(1) Fever: differentiate from systemic lupus erythematosus and sepsis. (2) Rash: differentiate from measles, scarlet fever, and erythema multiforme. (3) Lymphadenopathy: differentiate from infectious mononucleosis and leukemia. (4) Small joint redness, swelling, and pain: differentiate from juvenile rheumatoid arthritis.