enteropathic acrodermatitis in children
Introduction
Introduction to pediatric intestinal dermatitis Acrodermatitisenteropathica, a chronic intestinal dermatitis, also known as acrodermatitisenteropathicasyndrome, Brandt syndrome, Danbolt-Closs syndrome, enteric acral dermatitis, SARS Type-type continuous acral dermatitis, atypical bullous epidermolysis. It is an autosomal recessive hereditary disease characterized by scarring dermatitis around the mouth and extremities, repeated intermittent diarrhea, hair loss and paronychia. basic knowledge The proportion of illness: 0.03%--0.05% Susceptible people: infants and young children Mode of infection: non-infectious Complications: pediatric malnutrition
Cause
Causes of pediatric intestinal dermatitis
(1) Causes of the disease
Current research data suggest that zinc absorption in the intestine is a causative factor, and most cases have a positive family history.
1. Long-term lack of zinc: children treated with intravenous nutrition therapy, long-term lack of zinc, can occur rash and diarrhea similar to this disease, symptoms disappeared or reduced after treatment with zinc.
Lactating mice were used as animal models. After feeding the zinc-free food for several weeks, the skin scaly lesions were seen, and the hair was dry and shedding, which was similar to the symptoms of the disease. It was quickly effective after treatment with zinc.
2. Reduction of zinc absorption: The recipes in the Middle East contain more phytate, hinder the absorption of zinc, and produce dwarf pathology in the population with low sexual function, which is similar to the growth and development disorder seen in this disease.
3. After zinc supplementation, there is curative effect: the patient's food contains less zinc and has curative effect after zinc supplementation.
(two) pathogenesis
Some people think that there is a small molecule zinc ligand (Zinc-ligand) in human milk. Although the zinc content in human milk is not high, it is easily absorbed by infants, except for human milk, including milk. Other protein foods have a small peptide that easily complexes zinc in food to form a complex state of zinc that is not easily absorbed by the body. Usually, this small peptide is secreted by normal intestinal cells. Peptidase is hydrolyzed, and the patient often lacks such a short peptidase, so that the ingested zinc is complexed by small peptides, which affects the absorption of zinc and causes the deficiency of zinc.
Because zinc is related to more than 90 enzymes, the clinical manifestations of this syndrome are complex and diverse.
Skin biopsy is non-specific, may have hyperkeratosis, parakeratosis, thickening of the spinous layer, sometimes visible epidermis pustules, sponge formation, multinuclear leukocyte infiltration in the upper part of the dermis, no serious histology found in jejunal biopsy Changes, but with succinate dehydrogenase and leucine transpeptidase reduced, large and small intestinal biopsy specimens showed characteristic changes in the intestinal mucosa: villi flattened or disappeared, the intrinsic membrane showed inflammatory cell infiltration, epithelial cells became shorter, The nucleus is round or elliptical, located in the center of the cell but not in the normal position of the basal part of the cell, the nuclear chromatin is loose, and the ultrastructural abnormalities of eosinophils are characteristic. These intestinal mucosal lesions are often found in zinc preparations. After treatment, it gradually disappeared. Autopsy found mucosal erosion of the sigmoid colon and ascending colon and fatty changes of liver cells.
Prevention
Pediatric enteropathy apical dermatitis prevention
This disease is a hereditary disease. It should pay attention to a positive family history, strengthen genetic counseling, advocate breastfeeding after birth, and give zinc preparations early and long-term to prevent the occurrence of this disease.
1. The amount of zinc in colostrum is 6-7 times that of mature milk, so it should be opened early.
2. Prevent bad habits such as partial eclipse, picky eaters, and excessive snacks.
3, to supplement a variety of foods rich in zinc, zinc is mainly found in animal foods, plant foods and fruits are very low.
Complication
Complications of pediatric enteropathic acral dermatitis Complications, malnutrition in children
It means that the toenails are deformed or even missing, secondary lactose intolerance, malnutrition, stagnant growth and development, and may be combined with bacterial or fungal infections.
Symptom
Pediatric intestinal dermatitis symptoms common symptoms refers to (toe) nail loss pustule watery stool hair loss diarrhea herpes mouth ulcer glossitis
Onset
Most of them are under 1 year old, most of them have family history after weaning and have nothing to do with gender, race and season.
2. Rash
A variety of rashes appear, the rash begins as a herpes, quickly merges into a bullous, contains a serum, surrounded by red halos, there is a secondary rash in the form of pustules, rupture of the blister becomes flaky, red wounds, moss Samples or psoriasis-like plaques, tan dry scaly or crusted, such rashes appear in batches, stretch for a long time, no scars after disappearing, rashes occur in the skin mucosa junction area, and symmetry lesions can also appear in the limbs. Especially the extremities are severe, often involving the fingers, toenails, causing them to deform, atrophy, and even the loss of nails.
3. Mucosal inflammation
Visible stomatitis, glossitis, rhinitis, blepharitis, conjunctival keratitis and vulvitis.
4. Hair is dry and shedding
Forming a part of baldness or total baldness, even involving eyebrows and eyelashes.
5. Diarrhea
Most cases have varying degrees of diarrhea, and watery stools containing foam or mucus are thought to be due to secondary lactose intolerance.
6. Malnutrition
More crying, more sleep, stagnant growth and development, is a prolonged course of disease, when the time is good, the condition is getting worse.
Examine
Examination of pediatric intestinal dermatitis
1. Serum zinc: In most cases, serum zinc is reduced, often below 40 g/dl (normal value is 68-110 g/dl).
2. Urine zinc: The urine zinc content is reduced, and the normal value of children is 0.4 mg/d.
3. Zinc-containing enzymes: such as alkaline phosphatase activity is low.
4. Feces and skin culture: Candida is often positive, but not a pathogen and secondary infection.
5. If necessary, if you have a chest X-ray examination when you have a respiratory infection, you can do a bone X-ray to check the metaphysis and bone age when you are suspected of rickets.
Diagnosis
Diagnosis and diagnosis of pediatric intestinal dermatitis
diagnosis
Mainly based on clinical manifestations, blood zinc levels were significantly reduced, only 20 ~ 60g / dl, and the effect of zinc preparations after treatment was obvious.
Differential diagnosis
According to clinical manifestations, combined with age of onset and family history, diagnosis is not difficult, laboratory tests can assist diagnosis, but should be associated with extensive Candida infection, bullous epidermolysis, severe malnutrition and continuous acrodermatitis Identification of Candida infection, bullous epidermolysis in addition to bullae on the basis of systemic erythema, no other performance, and identification with intrinsic is not difficult.
