Pediatric Shi Yue syndrome
Introduction
Introduction to Pediatric Shi Yue Syndrome Pediatric Shi Yue syndrome is also known as mucocutaneous syndrome, Stevens Johnson syndrome, exudative erythema multiforme, malignant bullous erythema multiforme, Fuchs syndrome, etc. This syndrome is a more serious type of polymorphous exudative erythema syndrome, which mainly presents polymorphous skin lesions at different stages, mouth and eye mucosal lesions, and systemic poisoning symptoms. Basic knowledge Prevalence rate: 0.0002% Susceptible population: children Mode of infection: non infectious Complications: pneumonia, myocarditis, brain edema, hematuria
Cause
Etiology of Shih Yue syndrome in children
（1） Pathogenesis
The etiology of this syndrome is not completely clear, some people think it may be bacterial or viral infection, allergic disease caused by drugs.
（2） Pathogenesis
It is generally believed that it is the allergic reaction of the body to some antigens. The confirmed pathogenic factors are as follows.
1. Infection (bacteria, virus, mycoplasma): It often occurs after a cold, especially related to herpesvirus infection. Some patients are accompanied by pneumonia. The titer of serum condensates rises and mycoplasma pneumoniae is isolated.
2. Drug allergy: some people think that the disease belongs to severe drug eruption, and related drugs include penicillin, sulfanilamide and other antibacterial drugs; Butazone, salicylate, mercury, codeine, barbiturates, antiepileptics, etc.
3. Others: occasionally related to vaccination, endocrine disorders, malignant tumors, etc.
Prevention
Prevention of Pediatric Shi Yue Syndrome
We should actively prevent and control all kinds of infections, do a good job in the care of children's skin and mucous membrane, do a good job in vaccination, strengthen nutrition and strengthen physique. For allergic skin diseases, we should go deep and carefully to find allergens, avoid further contact, and avoid eating food related to the disease or aggravating the disease, such as fish, shrimp, crab and other foreign proteins, such as drug allergy, Try to find out the sensitization drugs, and explain the situation to the patients and their families. When they go to the hospital, they should also explain to the doctor to obtain cooperation, and no longer use the drug and drugs containing the drug ingredients that sensitize the drug.
Complication
Pediatric complications of Shigella Jorge syndrome complication Pneumonia, myocarditis, cerebral edema, hematuria
Hyperpyretic convulsion may occur, others may be complicated with otitis media, secondary bronchitis, pneumonia, arthritis, myocarditis, brain edema, liver damage, etc. If the kidney is involved, proteinuria, hematuria, urea nitrogen increase, etc. may occur, and in severe cases, intestinal mucosal ulcer bleeding, necrotizing pancreatitis may occur. In rare cases of myocarditis, circulatory failure often occurs in very serious cases, with poor prognosis.
Symptom
Symptoms of Shih Yue syndrome in children common symptom Mucosal damage, hoarseness, abdominal pain, high fever, diarrhea and scab Dysphagia, dyspnea, dysuria, salivation
This syndrome is mostly seen in youth and childhood, and its main clinical manifestations are as follows.
1. Symptoms of systemic poisoning
The disease starts with high fever, and the body temperature can be as high as 38~41 ℃. Infants can have febrile convulsions. The fever lasts for about 2~3 weeks, and skin and mucous membrane damage occur within 1~10 days. As the disease enters the extreme stage, the systemic symptoms gradually worsen. About 1/3 of the cases are accompanied by pneumonia. X-ray chest fluoroscopy shows patchy shadows. A few cases may have arthritis, and occasionally have myocarditis and glomerulonephritis, Circulatory failure often occurs in patients with severe diseases.
2. Extensive skin lesions
Erythema, blisters, typical bullous polymorphous erythema of different sizes, negative Nielsen's sign, occur repeatedly in the limbs, trunk, head and face, and even all over the body. In severe cases, they fuse into large areas. After blistering, a large amount of serous exudation and erosive surface appear as a second degree burn. If there is no secondary infection, scab and desquamation will occur 1 to 4 weeks later, leaving no scar, and the skin damage can be prolonged for 3 to 6 weeks.
3. Mucosal damage
(1) Gastrointestinal manifestations: almost all cases have stomatitis, which is characterized by bullae, erosion, bleeding and crusting, dysphagia and salivation due to pain, and a few cases have mucosal erosion in the stomach, small intestine and even rectum and anus, resulting in abdominal pain and diarrhea; The incidence of oral mucosa damage is 100% according to Wang Shanglan and other domestic and foreign data.
(2) Eye symptoms: Maureen et al. believed that the main early symptoms of the disease were acute conjunctivitis, periorbital swelling, and shyness caused by keratitis. In addition, there were corneal edema, iridocyclitis, and pus in the anterior chamber. The incidence of eye inflammation was 80%~90%.
(3) Urinary system performance: cystitis, vulva and urethro stomatitis cause dysuria.
(4) Respiratory manifestations: nasal vestibule, throat, trachea, bronchial mucosa erosion, hoarseness and dyspnea.
Examine
Examination of children with Shih Yue syndrome
Leukocytes increased, eosinophils increased, ESR accelerated, anti "O" value increased, and C protein reaction was positive.
Chest X-ray examination is to determine whether there is lung disease and pneumonia. X-ray chest fluoroscopy is patchy shadow. Other examinations such as B ultrasound, ECG and brain CT are selected according to clinical needs.
Diagnosis
Diagnosis and differential diagnosis of Shigella Jorge syndrome in children
diagnosis
The diagnosis can be made according to the clinical manifestation and the laboratory test results.
differential diagnosis
1. Pemphigus: After a slow process, the systemic toxic symptoms are mild, there is no inflammation at the base of the blister, Nielsen's sign is positive, and there are no blister cells in the cytological examination.
2. Drug dermatitis: Bullous erythema multiforme is similar to this disease, but it has a history of taking drugs and has a good prognosis.
3. Toxic epidermal necrolysis (Lell's disease): the symptoms are more serious than this disease, the onset is rapid, and the skin lesions are extensively exfoliated like a second degree scald. Because the epidermis is rapidly necrotic, the skin is dark purplish red, and Nielsen's sign is positive.
