mucocutaneous lymph node syndrome
Introduction
Introduction to skin mucosal lymph node syndrome Skin mucosal lymph node syndrome, also known as Kawasaki disease (Kawasakidisease), skin-mucosa-lymph node syndrome (MCLS), is an acute fever and rash disease in children. Its characteristic manifestations are: skin mucosal changes and non-suppurative lymphadenopathy, the course of disease is self-limiting, and a small number of patients may also die suddenly due to coronary artery disease. The disease was first proposed by Kawasaki (1961) and officially reported in 1967, and was named "acute (toe)-specific desquamation with acute febrile skin mucosal lymph node syndrome." The disease is widely distributed in Japan, and in recent years there have been many cases reported in China. basic knowledge The proportion of illness: 0.001-0.08% Susceptible people: infants and young children Mode of infection: non-infectious Complications: abdominal pain, nausea and vomiting, diarrhea, jaundice, arrhythmia, anemia, paralytic ileus
Cause
Cause of skin mucosal lymph node syndrome
(1) Causes of the disease
The etiology is still unclear. It is discussed from the aspects of clinical, pathological and epidemiological aspects. There are several the following theories:
Infection factors:
1. Streptococcal infection, such as the positive rate of polysaccharide antibody and T protein antibody of Streptococcus hemolyticus, is about 30%, while the control group is negative, and the purified polypeptide glycoside is extracted from the cell wall of hemolytic streptococcus for antigen-sensitized blood cell agglutination. Response, the disease titer is higher than rheumatic fever, scarlet fever is high, the application of hemolytic streptococcus bacterial extract for phytohemagglutinin test, its titer is also increased, the recent toxic shock syndrome caused by hemolytic streptococcal infection New clones of toxin products and pyrogenic exotoxins have shown that hemolytic streptococcus may be the cause of this disease.
2. Rickettsia, Chlamydia trachomatis infection rickettsia and Chlamydia trachomatis were found in biopsy specimens of rashes, so it is suggested that it may be related to this disease, and rickettsia has been successfully isolated from the blood.
3. Viral infections such as Epstein-Barr virus, adenovirus, human herpes virus, Coxsackie virus infection before the onset of the disease, some people have developed this disease after vaccination or taking polio vaccine, swallow swabs, stools and other cells The virus was isolated and found to be positive for human herpesvirus type 4, adenovirus type 3, coxsackie virus B3, B4 type, echovirus type 11, herpes simplex virus and hepatitis virus, but specific viruses for this disease, The separation has not been successful, and the relationship between them needs further study.
(two) pathogenesis
Immunological abnormalities: Immunopathological studies have found that immune abnormalities are involved in the pathogenesis of this disease and are associated with HLA-BW51. In the acute phase of the disease, polymorphonuclear granulocytes, circulating immune complexes and complement are found in the affected area.
1. There is a significant difference in serum IgE between the acute phase and the remission phase of the disease. It usually increases significantly at the initial stage, and gradually decreases with the remission of the disease. The hard edema manifested by this disease is caused by increased vascular permeability, which is considered to be related to type I metamorphosis. The reaction is related to IgE-mediated reaction. Due to the release of inflammatory mediators such as histamine, it can cause biological effects such as telangiectasia, increased permeability, smooth muscle spasm, and increased glandular secretion.
2. The role of immune complexes is mainly involved in the occurrence of systemic arteritis, which is similar to the Arthus reaction, that is, the acute serum disease response that is close to the excess antibody, due to the formation of immune complexes and activation of complement along the vessel wall. Inflammatory reaction causes vascular disease, especially the development of immune organs in children is not perfect, and it is not easy to intercept and clear immune complexes, so it is more likely to develop disease.
3. Using immunofluorescence technique, it was found that there were IgG in the intima of the myocardium, renal artery and lymphatic arterioles, and the island was reported to have anti-human albumin antibody, anti-fibrin fluorescent antibody, anti-human in the coronary artery. The IgG antibody fluorescence response is significantly enhanced, and it is found that there are anti-human IgG antibodies in the myocardium, suggesting that the immune complex precipitates to participate in the pathogenesis of vasculitis. These immune complexes can activate complement and attract leukocytes to the immune complex deposition. And accumulate at the deposition site, releasing lysosomal enzymes and various hydrolases to cause an inflammatory reaction, thereby causing necrosis of blood vessels and adjacent tissues in which the immune complex is deposited.
4. ANCA and anti-vascular endothelial cell antibody (AECA) can be detected in the serum of patients with acute phase, which may be involved in the pathogenesis of this disease.
The main pathological changes of Kawasaki disease are systemic vasculitis, especially coronary artery disease, including coronary aneurysm. In the acute phase, there may be vasculitis of moderate arteries (such as coronary artery, inter-renal artery, etc.). Vasculitis is caused by acute inflammation. Characteristics, sustainable for about 7 weeks, not necessarily accompanied by fibrinoid necrosis, the course of vasculitis can be divided into 4 phases, the first phase is the first 2 weeks of onset, microvessels (small arteries, capillaries, venules), Arterial and venous perivascular inflammation, which in turn affects the intima of the large and medium arteries, around the adventitia and perivascular, with edema, leukocyte and lymphocyte infiltration, and the second phase begins around the second week after the disease, lasting about 2 weeks. It is characterized by inflammation reduction of microvessels, aneurysm and stenosis in moderate arteries, especially coronary arteries, edema, mononuclear cell infiltration, increased capillary, granuloma formation, and stage 3 to 7 to 7 weeks after onset. Inflammation of microvessels and granuloma formation in moderate arteries were further attenuated, and after 7 to 8 weeks, they entered stage 4, in which moderate arterial scar formation, intimal thickening, aneurysm and stenosis, heart and Vasculitis of large and medium arteries such as arteries is more common. Occasionally, aneurysms are seen in other arteries such as mesentery and renal arteries. Vasculitis can also be seen in the arteries and veins of the heart, skin, kidneys and tongue, myocarditis, endocarditis, Cholangitis, pancreatitis, mumps, meningitis and lymphadenitis are also seen.
Prevention
Skin mucosal lymph node syndrome prevention
Primary prevention
Because the cause of this disease is unknown, it can only reduce the incidence by paying attention to nutrition, exercising the body, reducing the application of chemical drugs, and avoiding environmental pollution.
2. Secondary prevention
(1) Early diagnosis: The age is between 2 months and 5 years old. According to the clinical manifestations, combined with the diagnostic criteria of this disease, and other diseases are excluded, the disease can be diagnosed.
(2) Early treatment.
3. Three levels of prevention
(1) Complications should be prevented after the onset of illness. Kato and other scholars believe that the high risk factors for Kawasaki disease are:
1 giant coronary aneurysm, diameter 8mm, multiple, cystic;
2 fever has a long course of disease, more than 3 weeks;
3 had been treated with hormones;
4 The age of onset is over 2 years old;
5 anemia is severe, the reaction vasculitis is serious, control of the above factors can reduce coronary aneurysm rupture, myocardial infarction, thrombotic occlusion, cardiogenic shock, heart rhythm disorder, etc., is the key to improve the cure rate and reduce the mortality rate, the disease Most of the prognosis is good, the mortality rate is 0% to 2.6%, but there may be sudden death in the recovery period. There have been recurrence after 14 years of observation. It should be monitored to prevent the above high risk factors and recurrence.
(2) The children with this disease are more weak, belonging to the Chinese medicine to the yin deficiency body, should pay attention to the treatment to reduce the recurrence of the disease, but recurrence more than 3 times are rare, should be followed up for a long time.
Complication
Complications of skin mucosal lymph node syndrome Complications abdominal pain nausea and vomiting diarrhea jaundice arrhythmia anemia paralytic ileus
Acute gastrointestinal complications include abdominal pain, vomiting and diarrhea, gallbladder edema, mild jaundice, often complicated by arrhythmia, anemia, hyperalbuminemia, cardiac hypertrophy, and sometimes paralytic ileus and mild transaminase increase.
Symptom
Symptoms of skin mucosal lymph node syndrome Common symptoms Lymph node congestion, abdominal pain, diarrhea, relaxation, hot water, neck, lymph node enlargement, jaundice, hyperthermia, congestion block
1. The main clinical manifestations Kawasaki disease is an acute febrile disease, clinically can be divided into acute phase, subacute phase and recovery phase, often self-limiting.
1 acute fever period: often lasts for 1 to 2 weeks, characterized by fever, combined with membrane infection, oral mucosa erythema, redness of hands and feet, rash, cervical lymphadenopathy, aseptic meningitis, diarrhea, abnormal liver function, this period There may be myocarditis pericardial effusion, coronary arteritis.
2 subacute phase: 1 to 2 weeks after the onset of fever, rash and lymph node swelling gradually disappear, may have irritability, anorexia or mucosal infection, this period is characterized by peeling, thrombocytosis, coronary aneurysm rupture often in this period occur.
3 recovery period: 6 to 8 weeks after onset, all clinical symptoms disappeared until the erythrocyte sedimentation rate returned to normal.
(1) Fever: All patients have fever, can be missed heat or relaxation heat, often lasting more than 5 days, but most patients with fever can resolve within 3 weeks, body temperature can also slowly decline naturally, severe cases can also last for a long time Fever, bimodal fever, Sanfeng fever, severely ill patients can die of coronary artery disease, Kawasaki disease with sudden onset of fever, sometimes with a flu-like prodromal symptoms, sometimes without any prodromal symptoms, usually relaxation heat or heat retention, Can be as high as 39 ° C or more, if not treated often for 1 to 2 weeks, or even 3 to 4 weeks, if treated with aspirin and intravenous gamma globulin, 1 to 2 days can often be fever, antibiotics have no significant effect on fever.
(2) Limb changes: in the acute phase, symmetry from the hand, the back of the foot to the end of the finger (toe), diffuse non-depressed edema, erythema at the end of the palm, sole and finger (toe), from the onset of disease 2 From the weekend to the third week, membranous desquamation begins to appear at the junction of the nail bed and the skin at the end of the nail. This feature can be used as a basis for diagnosis. The change of the nail is called Kawasaki Henggou, which can be used with the finger (toe). The growth of nail A moves to the end, and the lateral groove reaches the end 10 to 12 weeks after the onset, and disappears after further development. This sign can be found in all cases.
(3) There are rashes such as urticaria, scarlet fever-like rash and childhood acute rash. Individual patients may also have typical exudative erythema. Generally, there is no itching sensation. The rash is more than the trunk. It can also be seen in the face and In the limbs, the BCG injection site can be obviously red and bloody, and the rash appears more than the third day after the onset of the disease, which lasts for 1 to 10 days and has no pigmentation after healing.
(4) Mucosal changes: from the early stage of the disease to the 10th day, the lips are severely congested and red, such as sputum-like fat, and the lips are covered with scaly or dry rupture during high fever. This is a specific change of the disease, oral cavity, pharynx and Tongue mucosa can also appear congestion, but there is no pus and pseudomembrane in the pharynx. The tongue can be strawberry-like due to congestion, which is similar to the tongue of scarlet fever, also known as "strawberry tongue."
(5) Ocular manifestations: conjunctival hyperemia, especially conjunctival hyperemia, the incidence is also high, can appear on the 3rd to 6th day after the onset, disappears by the second week.
(6) Non-suppurative lymphadenopathy: About half of patients may have cervical lymphadenopathy, unilateral or bilateral, which is a transient lymphadenopathy, which is most obvious on the 5th day after onset, with local tenderness, but No signs of suppuration are seen. The small arteries in the enlarged lymph nodes have intima, conjunctivitis, high edema of connective tissue around the blood vessels, and obvious inflammatory cell infiltration. Rickettsia is often found in the phagocytic cytoplasm around the blood vessels. Sample particles.
2. Cardiovascular system manifestations Cardiac involvement is the main feature of this disease. In the acute phase, more than 80% of patients have symptoms of myocardial inflammation. Myocarditis can appear in the first week, which is characterized by heart murmur, galloping, heart sound, and electrocardiogram. PR prolonged, STT changes, R wave voltage is low, chest X-ray shows heart enlargement, may be caused by myocarditis and (or) pericarditis, acute periventricular pericarditis can cause pericardial exudation, pericardial exudate is generally less, Self-dissipation, rarely caused by pericardial tamponade, in the acute phase due to myocardial lesions may appear congestive heart failure, in the subacute phase of heart failure caused by myocardial ischemia and myocardial infarction, valvular heart disease is rare, the affected valve is mainly the second tip valve.
About 20% to 25% of untreated patients may have coronary artery abnormalities. At the beginning of the fetus, a diffuse dilatation of the coronary artery can be measured by two-dimensional ultrasound diagnosis. The formation of coronary aneurysm can be measured at the first weekend of the disease. Usually peaks at 3 to 4 weeks, aneurysms with an inner diameter of less than 5 mm are called small aneurysms, those with an inner diameter of 5 to 8 mm are called middle aneurysms, those with greater than 8 mm are called large aneurysms, and those with acute arteritis are relieved. There is no chronic inflammation in the wall, small aneurysms may subside, large and medium aneurysms may remain unchanged or even stenosis, resulting in myocardial ischemia, more common in children than myocardial infarction, can occur in sleep or rest, the main symptoms are shock, Vomiting, restlessness, elderly children often have abdominal pain, chest pain, myocardial infarction of Kawasaki disease has a typical ECG change, fever is greater than 16 days, heat relapse 48h after recurrence of heat, I degree atrioventricular block, heart rhythm disorder, heart Large, low platelets, hematocrit and low plasma albumin.
Kawasaki disease vasculitis may also involve moderate arteries other than coronary arteries. About 2% of untreated cases may develop systemic vasculitis. The more commonly affected arteries have kidney, ovary, epididymis, mesentery, pancreas, ankle, and liver. , spleen and radial artery, these cases generally have coronary aneurysms.
3. Other clinical manifestations Acute gastrointestinal complications include abdominal pain, vomiting and diarrhea, gallbladder edema, mild jaundice, and sometimes paralytic ileus and mild transaminase increase.
In the acute phase, infants often have more irritability than other fever diseases. About 1/4 have aseptic meningitis, and cerebrospinal fluid leukocytes are 25-100/ml. The lymphocytes are mainly sugar, the sugar is normal, and the protein is slightly high. In addition, there are ear tympanic membrane hyperemia, ocular uveitis, although fever, rash, and lymphadenopathy have subsided in the subacute phase, but conjunctival congestion, irritability and anorexia still persist, neurological complications include facial nerve palsy, seizures, Ataxia, hemiplegia, etc.
Arthritis and joint pain account for about 1/3. In the acute phase, the small joints are involved. The weight of the large joints is mostly affected by the 2nd to 3rd week after the disease. It usually lasts for 2 weeks, and can last up to 3 months. The white blood cells in the arthritis synovial fluid are mainly neutrophils, and the white blood cells in the synovial fluid are less in the late-onset synovial fluid. Other musculoskeletal systems still have ankle arthritis, myositis and aseptic femoral head necrosis.
Urinary tract abnormalities have urethritis with aseptic pyuria, abnormal penile fistula, testicular- epididymitis, cystitis, prostatitis, acute renal failure, interstitial nephritis and nephrotic syndrome.
The clinical symptoms of pneumonia are not obvious, but the pneumonia changes on the X-ray.
4. Rare clinical manifestations of peripheral gangrene are rare and serious complications, due to peripheral ischemia, mostly at the onset of Kawasaki disease, more common in young non-Asian children within 7 months, often accompanied Giant coronary aneurysms, or peripheral aneurysms (especially the radial artery), although salicylic acid, intravenous gamma globulin, prostaglandin E or sympatholytic blockers and thrombolytic anticoagulant therapy, there are quite a few cases Need to intercept (toe), or even amputation.
Examine
Examination of skin mucosal lymph node syndrome
1. Blood routine and blood sedimentation
Most of them have neutrophils, nuclear left shift, accompanied by poisoning particles, eosinophils are generally 0.5% to 2%, hemoglobin and red blood cells are slightly decreased in the early stage of the disease, more than half of the patients in the second to third week of onset of platelet significant Increased, bone marrow megakaryocyte proliferation, this change is related to thrombosis and heart failure, early stage and recovery of platelet aggregation ability, accelerated erythrocyte sedimentation rate.
2. Urine routine
About one-third of patients have proteinuria, which is generally considered to be thermal proteinuria, occasionally cast, and urine culture is also positive, mostly Gram-negative bacilli.
3. Biochemical inspection
In some patients, acute bilirubin increased, transaminase increased, and C-reactive protein was strongly positive. In most patients, total protein decreased, A/G decreased, 2 and gamma globulin increased significantly, and some patients had elevated lactate dehydrogenase, which exceeded 500 U. .
4. Immunological examination
In most patients, total complement, immune complex and platelet agglutination factor increased, IgG, IgM, IgA generally did not change, anti-zero increased, and about 30% of patients were positive for ANCA and AECA.
5. ECG examination changes about 1/2 patients, such as ST segment decline, T wave low or inverted, low voltage and conduction block.
6. Echocardiography has about 50% of transient coronary artery dilatation, 20% to 30% of aneurysms, and 25% of pericardial effusions.
7. Cardiovascular angiography can observe the extent of distal coronary lesions and stenosis.
Diagnosis
Diagnosis and differentiation of skin mucosal lymph node syndrome
diagnosis
Diagnosis can be diagnosed if it has 5 of the 6 main symptoms and 3 of the 5 minor symptoms, plus a non-specific ECG abnormality and hematological changes.
Differential diagnosis
Scarlet fever
It is a streptococcal infectious disease. It is rare in patients 1 to 2 years old. The rash is a small miliary papule erythema. It is distributed in the neck, axilla, and groin, while the extremities are rare. Treatment with penicillin has significant effect.
2. Childhood nodular polyarteritis
There are often long-term or intermittent fever, rash manifested as erythema, nodular erythema and urticaria, cardiovascular system often manifests as hypertension, exudative pericarditis, congestive heart failure, abnormal ECG and gangrene of the extremities, etc. Heavier, combined with clinical symptoms and related examinations, identification is not difficult.
3. Rheumatic fever
There is evidence of a hemolytic streptococcal infection, the incidence of rash is less than 10%, unlike the distribution of skin lesions in this disease, it is prone to heart (myositis), etc., it is not difficult to identify.
4. Polymorphic exudative erythema
Occurs in young adults, have prodromal symptoms of upper respiratory tract infection, rash often accompanied by small blisters, mucosal lesions may have ulcers and pseudomembrane formation, especially in the skin and mucous membrane junction area lesions are obvious, rarely accompanied by neck The lymph nodes are enlarged and there is no coronary aneurysm.
5. Measles: Generally, the rash on the 4th day of fever, often begins after the facial ear, may have fusion, rash and fever, catarrhal symptoms and cough increase, the skin rash subsides after leaving a light brown pigmentation, the oral mucosa has Koplik Spot, Kawasaki disease rash in the trunk of the limbs, typical perineal rash is obvious, rash regression without pigmentation, both diseases can have hand and foot swelling, white blood cells, ESR increased in Kawasaki disease, measles without comorbidity when white blood cells are low.
6. Toxic shock syndrome: low blood pressure, and Kawasaki disease causes a decrease in blood pressure in cardiogenic shock. Some infections, such as staphylococcal infection with toxic shock, increase serum creatinine phosphokinase, and Kawasaki disease Then no.
