Characteristic flaky peeling of the toes
Introduction
Introduction Mucocutaneous lymph node syndrome (MCLS), also known as Kawasaki disease, is an acute febrile rash pediatric disease characterized by systemic vasculitis. In 1967, Japanese Kawasaki Fusuke doctor reported for the first time. Because this disease can cause serious cardiovascular disease, causing people to pay attention to it, the incidence has increased in recent years. In the hospitalized cases of rheumatic diseases in Beijing Children's Hospital in 1990, 67 cases of Kawasaki disease, 27 cases of rheumatic fever; the same information of 11 hospitals in other provinces and cities Kawasaki disease is twice as much as rheumatism. Obviously, Kawasaki disease has replaced rheumatic fever as one of the main causes of acquired heart disease in children in China. Kawasaki disease is currently considered to be an immune-mediated vasculitis that is temporarily included in the connective tissue disease.
Cause
Cause
The cause is not yet clear. The disease is a certain epidemic and landlord, the clinical manifestations of fever, rash, etc., presumably related to infection. It is generally believed to be a variety of pathogens, including Epstein-Barr virus, retrovirus, or Streptococcus, Propionibacterium infection. In 1986, the activity of reverse transcriptase in peripheral blood lymphocyte culture supernatants was reported to increase, suggesting that the disease may be caused by retrovirus. However, most studies did not achieve consistent results. In the past, it has been suggested that mycoplasma, rickettsia, and dust mites are the pathogens of this disease and have not been confirmed. Some people think that environmental pollution or chemical allergy may be the cause of the disease.
1. Gamma globulin: Studies have confirmed that early intravenous gamma globulin plus oral aspirin can reduce the incidence of coronary aneurysm in this disease, it must be emphasized that within 10 days after the onset of the disease, the daily intravenous infusion of the drug Globulin 400mg/kg, input for 4 to 4 hours for 4 consecutive days; plus oral aspirin 50 ~ 100mg / kg · d points 3 to 4 times, for 4 consecutive days, after the sputum to 5mg / kg · d.
2. Aspirin: Early oral aspirin can control acute inflammatory processes and reduce coronary artery disease, but no controlled studies have shown that aspirin treatment can reduce the incidence of coronary aneurysms. The dosage is 30-100 mg/kg per day, divided into 3 to 4 times.
3. Corticosteroids: Adrenal cortex hormones have always been considered to have a strong anti-inflammatory effect, which can relieve symptoms, but it is later found that corticosteroids are prone to thrombosis and prevent coronary artery disease repair and promote aneurysm formation. Therefore, it should not be used alone. Treatment with corticosteroids such as prednisone unless complicated by severe myocarditis or persistent high fever. Severe cases can be combined with prednisone and aspirin. In order to control the early inflammation of the disease, the response is generally not only corticosteroids.
Examine
an examination
Related inspection
Electrocardiogram two-dimensional echocardiography
Acute phase
Increased white blood cell count, up to 20 × 109 / L, with nuclear left shift; mild anemia; platelet increase in the second or third week of disease can reach more than 500 × 109 / L; ESR increased up to 60mm / hour Above, C-reactive protein is positive. Serum immunoglobulin, especially IgE increased, complement increased slightly; serum transaminase increased. According to the affected organs, other laboratory abnormalities still have proteinuria and pyuria; cerebrospinal fluid lymphocytes increased; electrocardiogram has low voltage, ST-T wave changes, PQ and QT interval length and arrhythmia; X-ray examination of heart enlargement; Two-dimensional echocardiography can be seen in coronary artery dilatation or coronary aneurysm formation, and most of the left and right coronary arteries are involved at the same time.
2. Bacterial, viral culture and infection serological examination
No positive findings. Some cases were positive for the anti-proteobacteria OX1q antibody or OX-X antigen, but the specific rickettsial serological test was negative.
Diagnosis
Differential diagnosis
Need to be identified with the following symptoms:
Scarlet fever: An acute infectious disease caused by infection with Streptococcus mutans that produces erythrotoxin, characterized by fever, sore throat, and generalized rash.
Polymorphic erythema polymorphic erythema: also known as exudative polymorphous erythema, is an acute inflammatory skin disease with complex causes. The rash is pleomorphic, often accompanied by mucosal damage, and its characteristic lesion is iris-like erythema. The disease is prone to spring and autumn and is prone to recurrence. The highest incidence rate is 10-30 years old.
Nodular vasculitis (n.dularvasculs): a small vasculitis of the skin that is mainly caused by lymphocytic infiltration. The clinical feature is that it occurs in adults, and small skin nodules and nodular surfaces occur repeatedly in the calves or feet. The skin color is normal or reddish. It is usually arranged along the superficial veins. It is mildly painful or tender, and generally has no systemic symptoms. The course of the disease can vary from several weeks to several months.
