lamellar ichthyosis
Introduction
Introduction to lamellar ichthyosis Lamellar ichthyosis is an epidermolytic keratosis ichthyosis, also known as bullous congenital ichthyosis-like erythroderma, and is an autosomal dominant genetic disease with high distortion rate. Clinically rare. Causes eyelids and lip valgus. After a few days, the film peeled off and the skin showed a wide range of chronic flushing. There were grayish white or taupe polygons or diamond-shaped large scales with central fixation and free edges. Often symmetry occurs in the whole body, with limbs flexing, elbow fossa, national fossa, armpits and vulva. Excessive keratinization of the palms, excessive growth of nails and hair. After birth or several months after birth, there may be generalized and limited damage. basic knowledge The proportion of illness: this disease is rare, the incidence rate is about 0.0003%-0.0005% Susceptible people: no specific people Mode of infection: non-infectious Complications: paraplegia
Cause
Cause of lamellar ichthyosis
(1) Causes of the disease
The disease is autosomal recessive, and studies have shown that its epidermal cell division rate is significantly increased.
(two) pathogenesis
The pathogenesis is still not very clear.
Prevention
Lamellar ichthyosis prevention
Prevention: Skin care should be strengthened to keep the skin moist. Eat more foods rich in vitamin A. Bathing should not be too frequent, soap should not be used too much, skin care oil should be used after bathing, it can protect the skin from softness, reduce scales, and maintain proper moisture and adequate nutrients.
Complication
Lamellar ichthyosis complications Complications
1 ichthyosis sputum sputum sputum intelligence syndrome <br /> Most patients develop neurological disease within 4 to 30 months after birth, mainly mental retardation, spastic quadriplegia or paraplegia. The lower extremity spasm is progressively aggravated, at least until puberty to relieve or stop progression. Most patients have similar lesions in the upper limbs, but are lighter. Most patients have mental retardation and do not increase with age.
2 intelligent disorder epilepsy ichthyosis syndrome <br /> infancy onset. It is mainly composed of mild ichthyosis-like erythroderma, mental disorders and epilepsy. However, some patients also have symptoms such as genital hypoplasia and dwarfism. The intelligence is very poor and you can't take care of yourself. Epilepsy is mostly a major episode. This disease may be caused by autosomal recessive inheritance, but it cannot exclude sexual chain inheritance.
Symptom
Symptoms of lamellar ichthyosis Common symptoms Skin striate scaly layered scales sputum palmar keratosis excessive palpebral erythema erythema (border clear...
At the time of birth or shortly after birth, you will see diffuse redness in your body. On the erythema, there are large patches of squares forming a square tan scale. The center is stuck and the edges are free. The scales around the large joints are often thick and thick, and the facial damage is particularly noticeable. Almost every case has a serious diagnostic valgus valgus, which causes scarring of the scalp due to frequent bacterial infections on the skin. There is a lot of severe hyperkeratosis in the palmar area, and the cleft palate causes obvious pain and hinders the function of the hands and feet. The nail is thickened and the surface is uneven. Because of gland blockage, it often leads to no sweat.
Examine
Examination of lamellar ichthyosis
1. Hereditary disease screening;
2. Histopathological examination
(1) Dominant hereditary lamellar ichthyosis: moderate epidermal hyperkeratosis with thinning or disappearance of the granular layer;
(2) Orthotopic lamellar ichthyosis: excessive keratinization, normal or slightly thick granular layer;
(3) Epidermolytic keratosis excessive lamellar ichthyosis: hyperkeratosis, epidermal cell release, particle degeneration.
Diagnosis
Diagnosis and identification of lamellar ichthyosis
Differential diagnosis
1, ichthyosis vulgaris: mainly in the extremities of the extremities and the trunk of the dry brown diamond, or polygonal scaly, the upper arm and thigh extension often have obvious follicular keratotic papules, palm toe involvement. The incidence rate is very high, and its keratinized scaly is caused by several layers of keratinocytes not falling off, more than several months after birth, the heaviest about 5 years old, the symptoms may be alleviated after puberty, but the disease is aggravated with age and treatment. .
2, sex-linked hidden ichthyosis: can occur after birth or infants. Skin lesions are large and prominent, yellowish brown or stained black large scales of scales, dry and rough skin, often throughout the body, axillary, and elbow fossa can also be affected; abdomen, back is particularly heavy. If the face is affected, it is limited to the front of the ear and the side of the face. Generally, follicular keratinization does not occur. The skin at the palm of the hand is normal, and the skin lesions do not decrease with age, but sometimes increase weight.
3, epidermis loosening hyperkeratosis ichthyosis also known as bullous congenital ichthyosis-like erythroderma: an autosomal dominant genetic disease with high distortion rate. Clinically rare. After birth or several months after birth, there may be generalized and limited damage. When the generalized person is born, there is a thick layer of scales in the whole body, which will fall off after birth, and there will be generalized flushing and scales. The scales will be removed, and the erythema will gradually disappear, and thicker scaly scales may occur. The situation limiter can have thicker scaly horny slices only in the flexor and wrinkle parts of the extremities.
