Pediatric purpura eczema syndrome
Introduction
Introduction to Pediatric Eczema Eczema Syndrome Aldrich syndrome (Aldrichsyndrome) is also known as eczema-thrombocytopenia-repetitive infection syndrome, Wiskett-Aldrich syndrome, Wiskett syndrome, Aldrich syndrome, Aldrich-Dees syndrome, eczema-thrombocytopenic immunodeficiency disease (immunodeficiencywitheczema- Thrombocytopenia), hereditary eczema thrombocytopenia immunodeficiency disease. The syndrome is characterized by repeated infections, bleeding symptoms caused by thrombocytopenia, and eczema-like lesions. In the early childhood, only seen in boys. basic knowledge The proportion of illness: 0.003% Susceptible people: children Mode of infection: non-infectious Complications: pneumonia
Cause
Causes of pediatric purple spot eczema syndrome
(1) Causes of the disease
The familial disease of this pathological attribute and recessive inheritance is transmitted to the male by the unaffected female, so it is only found in the male infant.
(two) pathogenesis
The genetic defect is that the polysaccharide or lipopolysaccharide antigen cannot be recognized and treated, and the stimulation of the microbial polysaccharide antigen has almost no ability to produce antibodies, so the child is susceptible to repeated infection, and there are also humoral immune abnormalities and cellular immune defects.
Platelets may have energy metabolism defects, resulting in platelet morphology abnormalities, shortened survival and aggregation, and the number of bone marrow megakaryocytes is normal or increased. Recently, platelet structural abnormalities and particle reduction, storage plate ADP reduction and platelets have been further proved. The release factor of factor 3 (PF-3) is reduced.
Prevention
Pediatric Eczema Eczema Syndrome Prevention
1. Find and remove the cause of the disease.
2. Feeding and diet
(1) Breastfeeding can reduce the extent of eczema. Protein supplements should be added later, such as eggs, fish, and shrimp. Generally, the baby is gradually added from 4 months, and the baby with eczema is recommended to be added 1 to 2 months later, and the rate of addition is slow. Your baby's diet should be as fresh as possible to avoid letting your baby eat processed foods containing gas, pigments, preservatives or stabilizers, and bulking agents.
(2) If you have found that a certain food has eczema due to consumption, you should try to avoid eating these foods again.
(3) Baby with milk allergy can be fed with soy milk, goat milk, etc. instead of milk.
(4) A baby who is allergic to eggs can eat egg yolk alone.
(5) Artificially fed babies suffer from eczema, which can be boiled for a few minutes to reduce allergies.
(6) Baby food should be light diet, should be less salt, so as not to have too much fluid in the body and easy to eczema.
3. Clothing
Cotton clothes can be used for the close-fitting clothes. All the collars are preferably cotton, and the clothes should be loose and soft. The bedding on the bed is preferably cotton, and the clothes, pillows, bedding, etc. should be replaced frequently and kept dry. Avoid overheating and sweating in daily life care. And let your baby avoid contact with allergens such as feathers, animal hair, pollen, and chemical fiber. It is not advisable to use silk, wool and chemical fiber products.
4. Bath and skin care
Bathing with warm water is best, avoiding the use of degreased alkaline toiletries and choosing acidic toiletries. Skin care products choose low-sensitivity or anti-allergic preparation skin care, and it is best to measure skin sensitivity to understand the skin's response to the skin care products used, and to prevent allergies in time.
5. Environmental aspects
The room temperature should not be too high, otherwise it will make the eczema itching worse. Minimize allergens in the environment to reduce allergic reactions caused by irritation.
6. Keep your baby's stool smooth and sleep well.
Complication
Complications of pediatric purple spot eczema syndrome Complications pneumonia
Digestive and urinary tract bleeding can occur. Skin lesions can be secondary to infection. Repeated bacterial and viral infections, such as pneumonia, chickenpox, etc.; malignant tumors that can be complicated by lymphatic network, 10% of patients with malignant lesions, such as lymphoma, reticulum sarcoma, retinocytic sarcoma, malignant net Histiocytosis and acute leukemia often die from severe bleeding, infection, and malignant tumors.
Symptom
Pediatric Eczema Eczema Syndrome Symptoms Common Symptoms Skin mucosa purpura ecchymosis repeated infection bleeding tendency gastrointestinal bleeding keratitis nasal bleeding thrombocytopenia eosinophilia
Hemorrhagic tendency
Children may have purpura and ecchymosis several hours after birth or in infants and young children. There are often bleeding after trauma or nose bleeding, mucosal bleeding, digestive tract and urinary tract bleeding. Some scholars believe that gastrointestinal bleeding may be The first symptoms of the disease, these bleeding symptoms are caused by thrombocytopenia or functional abnormalities.
2. Eczema lesions
Eczema lesions often occur 2 to 3 months after birth, occur in the head, limbs and other parts of the limbs, the shape of atopic dermatitis or seborrheic dermatitis, some skin lesions more bleeding or bleeding Secondary infections often fail for a long time.
3. Repeated infection
Children can have a variety of bacterial and viral recurrent infections, such as pneumonia, otitis media, herpetic keratitis, pyoderma, chickenpox, etc., susceptible pathogenic microorganisms and fungi, Pneumocystis carinii.
4. Laboratory inspection
Anemia (mostly iron deficiency, a small number of children may have hemolytic anemia, positive for Coombs test), platelet and lymphocyte reduction, eosinophilia, platelet aggregation of ADP and collagen, platelet decrease, life expectancy Shortening, low glycogen content, decreased activity of hexokinase (HK), increased -globulin, decreased IgM, and increased IgA and IgE.
The DNCB (dinitrochlorobenzene) skin test may not have a positive delayed response.
Examine
Examination of pediatric purple spot eczema syndrome
Immunological examination
Both children's cells and humoral immunity were defective, and the skin delayed allergic reaction was negative. About 90% of the children had negative skin test for dinitrochlorobenzene (DNCB), and the lymphocyte conversion rate was low after specific antigen stimulation, but after PHA stimulation. Lymphocyte transformation rate is normal, common blood lacks IgM and IgM genus antibodies (such as blood group cluster lectin, etc.), but IgA and IgG levels are normal or elevated, for polysaccharide antigens (such as pneumococcal capsular antigen ) After stimulating the body, the antibody is poor, the response to protein antigen is normal, the lymphocytes in the blood are often reduced, the complement content and phagocytosis are normal, and the thrombocytopenia is caused by the rapid destruction of platelet internal defects. The platelet volume is small, for ADP. , collagen and adrenaline have abnormal agglutination reactions.
2. Histopathological examination
In children, the number of small lymphocytes in the thymus is reduced, the cortex and medulla are difficult to distinguish, the lymphocytes in the paracortex of the lymph nodes are progressively attenuated, and lymphoid follicles are present.
According to clinical needs, choose X-ray chest X-ray, B-ultrasound and other tests.
Diagnosis
Diagnosis and diagnosis of children with purple spot eczema syndrome
diagnosis
According to the clinical manifestations and laboratory tests, DNCB skin test and other data, comprehensive analysis to make a diagnosis, bone marrow examination does not help the diagnosis, only the number of megakaryocytes is normal or increased.
Differential diagnosis
Different from the hemorrhagic disease caused by coagulopathy, clinical characteristics and laboratory results can help identify.
1. Hereditary coagulopathy is generally a single clotting factor deficiency, mostly bleeding symptoms in infants and young children, often family history.
2. Acquired coagulopathy is more common, patients often have a variety of clotting factors, mostly in adulthood, clinically in addition to bleeding with the symptoms and signs of the primary disease.
