alligator baby syndrome
Introduction
Introduction to crocodile skin infant syndrome A1ligatorbabysyndrome, also known as Carini syndrome, Seeligman syndrome, lamellar ichthyosis syndrome, neonatal stratified epidermal exfoliation syndrome, congenital ichthyosis-like erythroderma, collapionbaby Wait. This condition is a special type of infant with lamellar ichthyosis, characterized by rhomboid or polygonal taupe keratin scales and erythroderma. basic knowledge The proportion of illness: 0.001% (with family history) Susceptible people: children Mode of infection: non-infectious Complications: sepsis
Cause
Causes of crocodile skin infant syndrome
(1) Causes of the disease
Most of the symptoms are autosomal dominant or recessive hereditary diseases, and may also be associated with sexual inheritance.
(two) pathogenesis
The erythroderma is caused by a marked increase in the rate of epidermal cell division. Most of the mechanism of the disease is caused by a physiological variation or an increase in keratinocyte cohesion. Bowen believes that it is a persistent disease. Epidermal layer, similar to certain mammals.
Prevention
Crocodile skin infant syndrome prevention
Refer to the preventive measures of hereditary diseases, attach importance to the consultation of genetic diseases, and do a good job in preventive health care during pregnancy.
Complication
Alligator skin syndrome complications Complications sepsis
Can be accompanied by eyelid valgus, limited body position, skin infection or sepsis, often resulting in infant death.
Symptom
Symptoms of crocodile skin infant syndrome Common symptoms Peeling scales, scallops, red scales, abnormal scales
Most of the diseases are premature infants and low birth weight infants. Premature infants are live infants with a gestational age of 28 weeks to less than 37 weeks. Low birth weight infants refer to a body weight of 1000 to 2500 g. The skin is affected before birth. It is often stillbirth or early death after birth, so most of the typical skin lesions appear after a period of birth, not at birth, the disease has a good prognosis, the infant can cover the horny thick armor-like scales. Often accompanied by malformations and hypoplasia.
The symptoms of typical skin lesions gradually appear after hours or days of birth. The whole body or part of the skin is bright, dark red and thick. It seems to have a transparent film like plastic film, like crocodile skin or fire-cotton glue. After a few days. Gradually dry and large pieces fall off, can be repeated, because the body is covered with a transparent film, so that the limb is fixed in a special position, can be accompanied by eyelid eversion, new tender skin is easy to break, severe skin peeling infection, or even occur septicemia.
Children may also have stratified ichthyosis, abnormal ichthyosis or ichthyosis-like erythroderma.
A case report of fire-cotton gum: female, 2 days of normal delivery, estimated to be 37-38 weeks of pregnancy, weight 2700g, the history of the close relatives of the parents is uncertain, the skin of the child is brownish-yellow, large-area chapped, showing edge cocking, The opaque block, the nasal cavity and other cavities are filled with keratinous fragments, the eyes are valgus, the bilateral auricles are wrinkled, the face is tense, the expression is less, the upper limbs are semi-flexed, and the rest are not abnormal.
Examine
Examination of crocodile skin infant syndrome
1. Blood examination: Peripheral blood white blood cell count and neutrophil count were significantly increased in concurrent infection.
2. Blood test: When the serious infection occurs, attention should be paid to the liver, kidney function test, blood electrolyte and blood pH check.
3. Histopathological examination: significant keratinization, granules, thickening in some areas, thickening of the acanthosis, prolongation of the epidermis, keratinization in the hair follicle, chronic inflammation infiltration around the upper cerebral vessels.
Select the necessary auxiliary tests according to your clinical needs.
Diagnosis
Diagnosis and differentiation of crocodile skin infant syndrome
According to clinical typical skin lesions, skin biopsy, and pathologically visible keratohemorrhagic detachment of the stratum corneum can make a diagnosis.
According to the clinical characteristics of the disease, it is easy to distinguish it from other ichthyosis diseases. The diseases that should be identified are as follows:
1 septic erythroderma;
2 to distinguish between no bullous lesions and epidermolytic hyperkeratosis;
3 ugly, ugly is a serious type of fire cotton rubber, histopathological examination can help diagnose.
