lamellar ichthyosis
Introduction
Introduction Lamellarichthyosis, also known as recessive genetic congenital ichthyosis-like erythroderma, is a rare hereditary keratoderma that belongs to autosomal recessive inheritance. The skin of patients with ichthyosis is relatively dry, and should avoid excessive bathing in peacetime, especially if it is not washed with hot water or washed with soap that is too alkaline. When the winter climate is dry, pay attention to the oily skin care products to keep the skin moisturized. Pay attention to eating more vegetables and fruits, and eat some animal foods such as liver and carrots.
Cause
Cause
The disease is autosomal recessive. Studies have shown that the epidermal cell division rate is significantly increased.
Examine
an examination
Related inspection
Skin elasticity test skin test physical examination of skin disease
The typical symptom is that the whole body covers a large dark black or brown ichthyosis. Most of the newborn babies have the appearance of CollodionBaby. The outer layer begins to peel after the outer layer falls off. The affected part is whole body or limited to the head, abdomen and legs. Department, redness and unequal degree of desquamation, the dandruff is large, brown or gray, and tightly attached to the skin. In severe cases, hard dandruff will limit the movement at the wrinkles, palms, soles, and fingers due to the rotation of the limbs, and the joints will gradually become stiff.
Other symptoms include thickening of the hands and feet, thinning of the hair, valgus of the eyelids, finger deformities, and skin damage resulting in abnormal sweat glands. The patient's skin condition does not easily improve with age, but the life span is comparable to that of the average person. Because the skin of the newborn is destroyed, there is a risk of excessive water loss, electrolyte imbalance, body temperature imbalance and infection. Therefore, the humidity and temperature of the patient's environment must be properly controlled, pay attention to electrolytes and liquids, calorie intake, and prevent infection.
Diagnosis
Differential diagnosis
Different diseases of layered ichthyosis:
1, ichthyosis vulgaris: mainly in the extremities of the limbs and the trunk of the dry brown diamond or polygonal scaly upper arm and thigh extension often have obvious follicular keratotic papules. The incidence of palm toe is very high, its keratinized scale is Several layers of keratinocytes do not fall off and accumulate more than the number of months after birth appear around 5 years old. The most severe symptoms after puberty may be alleviated, but with the increase of age and treatment worse.
2, sexual chain cryptic ichthyosis: can be after birth or infantile lesions scales large and significantly yellowish brown or stained black large scale fish scales dry and rough skin often spread throughout the body armpits and elbow fossa and other parts can also be affected; If the face is affected, it is limited to the front of the ear and the side of the face. Generally, the hair follicles are not keratinized. The normal skin lesions of the skin do not decrease with age, but sometimes increase weight.
3, epidermis loosening hyperkeratosis ichthyosis: also known as bullous congenital ichthyosis-like erythroderma: for the automalignant genetic disease with high distortion rate clinically rare after birth or several months after birth can have pan The generalized condition and the limited nature of the lesions are generalized when the body is born, there is a thick layer of scales, and then there is a generalized flushing and scale stripping scales. The erythema can gradually disappear and can be re-growth. The scaly condition limiter may have thicker scaly keratin slices only in the flexor and wrinkle parts of the extremities.
4, layered ichthyosis: autosomal recessive inheritance after the whole body is a wide range of human cotton gelatinous membrane tightly wrapped more than the eyelids and lip eversion several days after the film peeling skin is widely diffuse flushing There are gray-white or taupe polygons or diamond-shaped large scales. The central fixation edge is often symmetrical. The symmetry occurs in the whole body. The limbs are flexed to the elbow fossa and the vulva and the vulva are more obvious. The palmar hyperkeratosis and hair overgrowth are slow and can last a lifetime. The presence of erythrodony in adulthood can be alleviated but the scales are still present.
5, scale-like red skin: autosomal dominant inheritance, a type of ichthyosis, and divided into blister type and non-blister type. The blister type is characterized by flushing of the whole body, showing herpes and large exfoliation of the epidermis, and the newly formed stratum corneum on the exfoliated surface is covered with large pieces of taupe scales. The non-blister type shows thickening of the skin and the skin of the child is red. The whole body is covered with a thick layer of cornea, which is shaped like a fire-cotton glue. After the capsule is removed, the red skin is left and large scales appear.
6, often ichthyosis: ichthyosis, is a common hereditary skin keratosis, formerly known as ichthyosis, Chinese medicine called snake skin. Mainly manifested as dry or rough skin on the extremities or trunk of the extremities, with rhomboid or polygonal scales, the appearance of which is like fish scales or snakeskin. Due to different genetic methods, it can be divided into autosomal dominant ichthyosis, sex. Inherited ichthyosis, congenital ichthyosis-like erythroderma and lamellar ichthyosis.
