enteropathic dermatitis syndrome
Introduction
Introduction to enteropathy dermatitis syndrome Enteropathic Acrodermatitis is a rare chronic skin disease. In 1936, it was first reported by Brandt, so it was called Brandt syndrome. In 1942, Danbolt and Closs II described it in detail, so the double name is Danbolt. -Closs syndrome, the main features of this disease are skin erosion, alopecia, nail malnutrition and paronychia, gastrointestinal dysfunction. This disease often occurs in infants from 3 weeks to 18 months. basic knowledge The proportion of illness: 0.0005%-0.001% Susceptible population: 3 weeks to 18 months baby Mode of infection: non-infectious Complications: angular cheilitis stomatitis conjunctivitis paronychia
Cause
Etiology of enteropathic dermatitis syndrome
Cause of disease:
The cause is still unclear, probably due to insufficient secretion of pancreatic enzymes, and some have pointed out that it is recessive inheritance. In recent years, studies have shown that hereditary zinc deficiency has a certain relationship with the occurrence of this disease.
It may be due to poor absorption of zinc, which may affect the structure or activity of certain enzymes, resulting in certain metabolic disorders, such as metabolic disorders such as tryptophan or fatty acids, which produce toxic substances, which may cause the disease.
There was no specific change in skin biopsy, and blistering under the skin layer was observed. The spine cells were thickened and edematized. Neutral polymorphonuclear and eosinophil infiltration were observed around the blood vessels.
Prevention
Enteropathic dermatitis syndrome prevention
Preventive measures: pay attention to zinc supplementation to prevent zinc deficiency. At the same time, we should actively prevent the occurrence of infection. Combined with a series of complications such as disseminated herpes, herpetic meningitis, prostatitis, proctitis, pelvic inflammatory disease, spinal nerve root disease. In areas where AIDS is endemic, the disease can also increase the risk of HIV infection. In addition to the symptoms of burning, erythema, and pain in the genital area, there may be general malaise, fever, headache, general joint and muscle pain.
Complication
Enteropathic dermatitis syndrome complications Complications, keratitis, stomatitis, conjunctivitis, paronychia
Rhinitis, angular cheilitis, stomatitis, glossitis, conjunctivitis, blepharitis, paronychia, nail atrophy, and decreased resistance, etc., easily secondary to systemic or local infections, often secondary to Candida albicans and staphylococcal infections.
Symptom
Symptoms of enteropathic dermatitis syndrome Common symptoms Loss of appetite, abdominal distension, glossitis, gastrointestinal symptoms, diarrhea, mild skin, scarring, hair loss
1. Skin damage: The original rash is blister, the blister fluid begins to become transparent, and later becomes cloudy due to secondary infection, the skin lesions fuse with each other to form patches of different sizes, surrounded by blush, symmetrically distributed in the skin and mucous membrane opening In the Ministry and around, other common parts are the hands and feet (especially around the nails), the wrinkles (front of the neck, the inside of the thighs, etc.), elbows, knees, head, eyes, nose, etc., after the skin lesions, the affected parts are dry, flushing , exfoliation, psoriasis-like appearance, clear boundary.
2. Gastrointestinal symptoms: About 90% of patients have gastrointestinal symptoms, mainly diarrhea, 3 to 6 times a day, often a large amount of grayish white with mucous fat stools, in addition, there is still loss of appetite, abdominal distension and so on.
3. Hair loss: hair is sparse or completely off, eyebrows, eyelashes can also fall off, to adulthood, even mane, pubic hair, mane do not grow.
4. Others: Some patients still have growth retardation, mental retardation, mental apathy, rhinitis, angular cheilitis, stomatitis, glossitis, conjunctivitis, blepharitis, paronychia, nail atrophy, and decreased resistance, etc. Secondary to systemic or local infections.
The above symptoms are easy to recurrent, good or bad, often secondary to Candida albicans and staphylococcal infections, Candida can often be cultured in feces and skin lesions, but dermatitis does not improve after treatment with antifungal drugs.
Examine
Examination of enteropathy dermatitis syndrome
Tissue biopsy: There is no specific change in skin biopsy. Blisters under the skin can be seen, and then the spine cells are thickened and edematous. Neutral polymorphonuclear and eosinophil infiltration can be seen around the blood vessels.
Diagnosis
Diagnosis and diagnosis of enteropathy with cutaneous dermatitis syndrome
Pay attention to the identification of psoriasis, herpes and so on. It can occur in any part of the body, but it is more common in the lower back. Herpes zoster is caused by filtering virus, trauma, syphilis, spinal cord disease, and arsenic poisoning. At the beginning of herpes zoster, the affected part often has itching, burning or painful feelings, sometimes accompanied by pre-symptoms such as general malaise, fever, loss of appetite, and herpes zoster virus often manifests as chickenpox after the initial primary infection, usually in the presence of In children, herpes zoster is caused by repeated or secondary infection. When varicella is attacked, a knife-like pain (neural pain) occurs, and a blister-like rash is caused on the skin of the pathogenic nerve. There are new rashes, new and old rashes are distributed according to the nerves, arranged in a band shape, the skin between the rashes is normal, and the herpes zoster affects the facial nerves. The patient may have facial paralysis. If the eyes are affected, the cornea will be damaged.
