lymph node congestion
Introduction
Introduction Mucosal lymph node syndrome (MCLS), also known as Kawasaki disease, was first reported in 1967 by Kawasaki of the Japanese Red Cross Center, and is also known as Kawasaki syndrome. This syndrome occurs in children and is currently unknown. It is an independent acute infectious disease. The main clinical manifestations were persistent fever, conjunctival hyperemia, lip flushing and cleft palate, hard redness of the hands and feet, pleomorphic rash of the whole body and enlarged lymph nodes in the neck, and lymph node congestion. Some patients develop arthritis and cardiovascular disease after the acute phase, and severe cases can die from myocardial infarction.
Cause
Cause
The cause is not clear, and it is speculated to be related to infection. It is generally believed to be a variety of pathogens, including Epstein-Barr virus, Coxsackie virus, Echovirus, adenovirus, retrovirus or streptococcus, and Propionibacterium infection. Some people think that environmental pollution or chemical allergy may be the cause of the disease. Epidemiological data and clinical features strongly suggest that mucosal cutaneous lymph node syndrome is associated with a certain infectious disease cause. It has been suspected to be Streptococcus, Staphylococcus aureus. Infections caused by secondary bodies, P. acnes mutants, and retroviruses have not been confirmed by standard bacterial, viral cultures and extensive sero-epidemiological investigations.
Mucosal cutaneous lymph node syndrome is prevalent throughout the world, with a group outbreak every 3 to 6 years, with the highest incidence in Japan and the United States. There have been cases reported in China since 1975, and there have been hundreds of cases so far. MCLS can occur in infants and children, but 80% to 85% of patients are within 5 years of age, and occur in infants between 6 and 18 months. The ratio of male to female is 1.5 to 1.8:1. There is no obvious regional and seasonal, and it is mostly distributed. The route of transmission is not clear.
Pathogenesis: The pathogen of mucosal cutaneous lymph node syndrome is unknown, so the pathogenesis is difficult to elucidate. Studies have shown that the role of superantigen is the cause of mucosal skin lymph node syndrome. The superantigen reaction is different from the classical antigen-antibody reaction. It is characterized by a large number of immune cells involved in some bacterial exotoxins, including toxic shock syndrome toxin 1 (TSST-1) produced by Staphylococcus aureus, staphylococcal enterotoxin. A to E, Streptococcus thermotoxins A, B and C, Mycoplasma toxins and certain retroviruses can bind to major histocompatibility sites of mononuclear macrophages and then bind to T cell receptors A special part of the -chain variable region (TCR-V), which can significantly activate mononuclear macrophages and selectively stimulate specific T cells to clone and proliferate, releasing excess Cytokines, including IL-1, IL-2TNF- and IFN-, cause systemic vascular endothelial cell injury. Melish found that TCR-V2 and TCR-V3 were over-expressed in patients with acute Kawasaki disease, and the recovery period turned normal. . Leung et al. cultured specimens from 16 patients with Kawasaki disease in the sacral rectal axilla and groin, and obtained 13 bacterial toxins, 11 of which were TSST-1 produced by Staphylococcus aureus, and 2 were pyrogenic toxins produced by Streptococcus. B and C. Therefore, the superantigen reaction that causes mucosal skin lymph node syndrome is considered to be mainly bacterial toxins, especially TSST-1.
In the acute phase of mucosal skin lymph node syndrome, serum total complement and C3 levels decreased, and the immune complex test showed a strong positive reaction. These changes gradually recovered during the recovery period. Intravenous infusion of large doses of human gamma globulin can significantly reduce vascular inflammatory response and prevent coronary artery expansion. All of the above phenomena support the pathogenesis of mucosal skin lymph node syndrome, and the mechanism is immune damage induced by certain pathogenic factors.
Examine
an examination
Related inspection
Blood routine urine M-mode echocardiography (ME) Doppler echocardiography two-dimensional echocardiography
Clinical manifestation
1. Acute phase (heating period): lasts 8 to 12 days, with an average of 10 days.
(1) fever: sudden fever body temperature 38 ~ 41 ° C, mostly relaxation heat, occasional heat retention; low appetite, lethargy or irritability, emotional excitability, severe cases of aseptic meningitis. Some patients may have diarrhea or obstructive jaundice. High heat generally lasts 5 to 11 days or longer.
(2) bilateral conjunctival hyperemia: conjunctival hyperemia can occur shortly after fever, and a small number of follicular conjunctivitis occurs.
(3) Oral mucosal changes: mouth flushing, cleft palate, diffuse oropharyngeal flushing, bleeding tongue nipple protrusion, redness, showing "yangmei tongue"-like changes.
(4) Skin rash: rash occurs 3 to 5 days after the disease, and the rash has more trunks, which can also occur on the face and limbs. The skin lesions were measles-like, scarlet-like, and erythematous, but no herpes and no pigmentation after the healing. The rash lasted for 1 to 2 days to 10 days. The rash has different distribution patterns, most commonly a pan-hair style, but sometimes the trunk is more pronounced, or peripherally distributed, and some are mainly in the perineum. In the acute phase, about 20% of cases have flushing and desquamation of the perineal and anal skin, and erythema or scarring is reproduced from the site of BCG vaccination 1 to 3 years ago.
(5) Limb lesions: 4 to 7 days after fever, large erythema appeared in the palm and ankle, the hand and foot showed hard edema, and the finger (toe) showed a fusiform swelling, often with limited joint activity due to severe joint pain. Limb lesions are characteristic features of MCLS, with a diffuse purplish red color in the palmar sac; in the recovery phase, characteristic desquamation occurs on the fingertips, often involving the entire palmar.
(6) lymphadenopathy: only seen in 50% of patients, cervical lymph nodes, mostly unilateral. The swollen lymph nodes are hard, 1.5cm in diameter, red and hot, and slightly tender, showing acute non-suppurative lymphadenitis.
2. Subacute phase: In this period, the main manifestations are large peeling of the palm and ankle, and the membranous desquamation begins at the end of the nail (the toe) and then the body is desquamated. Some patients develop arthritis and severe heart involvement symptoms. This period lasts about one month.
3. Recovery period: enter the recovery period from the sixth week of the disease course. In this period, all clinical symptoms gradually disappeared, and the blood and erythrocyte sedimentation rate returned to normal; in the later stage of recovery (2 to 3 months after onset), the nails appeared in some cases.
Diagnosis
Differential diagnosis
Need to be identified with the following diseases:
1, scarlet fever: 1 day after the disease, rash, diffuse small dense erythema, skin wrinkles at the rash more dense, visible dark red dot-like lines, extremities rash on the extremities, antibiotics effective.
2, toxic shock syndrome: the age of onset is relatively large, more common in menstrual women, low blood pressure (systolic blood pressure 90mmHg).
3, children with nodular polyarteritis: clinical often have long-term or intermittent fever, rash is erythema, urticaria or polymorphic erythema, may have high blood pressure, pericardial exudation, heart enlargement congestive heart failure and extremities Gangrene and so on.
4, juvenile rheumatoid arthritis: mostly low fever, repeated appearance of various forms of rash (hot rash rash), repeated heat, prolonged, non-finger, toe end redness (swelling pain in the center of the joint), no palmar flush , lips flushing, cleft palate, oropharyngeal mucosal congestion, bayberry tongue, no coronary damage.
5, scarlet fever: scarlet hot rash appeared 24 hours after the onset of the disease, the disease rash began on the third day after the onset of the disease, the rash appeared earlier than the disease; scarlet fever rash is a small needle small papule, the disease rash morphology close to measles and polymorphism Red spot; scarlet fever can be seen in all age groups, the age of this disease is infants and young children; scarlet hot throat swab culture has hemolytic streptococcus growth, anti-"O" titer increased, the disease is negative for these two tests; Scarlet fever with penicillin treatment is good, the disease penicillin has no effect.
6. Infectious mononucleosis: persistent fever, lymphadenopathy and Kawasaki disease have similarities, but no conjunctival hyperemia and oral mucosal changes, no hard swelling and peeling at the extremities. Peripheral blood leukocytes are classified mainly by monocytes, accounting for 70%-90%, and abnormal lymphocytes are up to 10%.
