Ichthyosis

March 20, 2012

Related Articles:

• Foot Fungus
• Price Klenzita
• Correction of facial aging changes
• Menovazin

Tweet

Ichthyosis   - A skin disease, which is characterized by a violation of keratinization of the skin. It refers to a group hereditary dermatological diseases . Etiology of ichthyosis is not fully understood, it is only known that the disease is caused by biochemical defect group of mutant genes . At the same time several different clinical forms of the disease, depending on the group of mutated genes.

Ichthyosis accompanied by failure vitamin A . hypogonadal . Thyroid . The pathological process recalls ichthyosis psoriasis But more aggravated. Development hyperkeratosis ichthyosis when leads to the scales on the skin similar to fish scales, which specifies the name of the disease.

According to the degree of skin lesions - keratinization, dermatological distinguish about 28 different forms of ichthyosis, as well as several rare syndromes in which acts as a symptom of ichthyosis. It is a symptom Ore syndromes . Sjogren-Larsson . Refsum   and etc. There is a variety of expressions hyperkeratosis   mild roughness of the skin, to strongly expressed pathological changes, often incompatible with life.

Since all the major forms of the disease have hereditary genesis , They are differentiated into two groups autosomal dominant type   inheritance recessive type   - X-linked ichthyosis . Depending on the group of diseases of different clinical manifestations, the complexity of the flow prognosis. The first group is found equally often both men and women, and the second only in men that is caused by a type of mutant genes.

The symptoms of ichthyosis

Among all forms of ichthyosis ichthyosis occupy 80-95% of autosomal dominant inheritance. The most common form - ichthyosis vulgaris Which is actually divided into several types.

The symptoms of ichthyosis characterized by diffuse skin lesions of trunk and extremities, which have varying degrees of severity. There are layers of different color and the form of flakes, the skin becomes rough and dry. The scales have a whitish color from nearly transparent to gray-black. The highest severity of lesions in places extension of limbs - the knees and elbows. At the same time the surface of the neck and bending elbows and knees are not affected.

Also in the clinical development of ichthyosis acts keratosis pilaris . At the mouth of the hair follicles formed small nodules dry hair becomes thin, sparse, brittle. Facial skin most often affects adults, there is peeling of the cheeks and forehead. Palms and soles have pronounced skin pattern, strongly expressed mukovidnoe peeling.

By kninicheskim featured ichthyosis vulgaris are divided into several types. Xeroderma   - Distinguished mild, characterized by dryness and light roughness of the skin, mainly on the extensor surface of the extremities. A simple form of ichthyosis   - Changes in the skin covers the whole body, legs and hairy head area.

The symptoms of ichthyosis is a simple type of small flakes, which are attached to the base of the central portion. Ichthyosis also differ: white   (white flakes, mukovidnye); brilliant   (transparent flakes in a mosaic localized on the limbs); serpentine   (large grayish-brown scales arranged like a serpent cover).

Most often, all of the above types are regarded as ichthyosis vulgaris in varying degrees of severity. At puberty ichthyosis symptoms subside later resumed. The disease lasts a lifetime, without moving from one form to another, as a rule, exacerbation occur during the cold season.

Histological diagnosis of ichthyosis vulgaris also reveals recessive ichthyosis . In the course of genetic research has been allocated to this group of previously identified ichthyosis vulgaris ichthyosis. The disease manifests itself fully only in boys.

Recessive ichthyosis X-linked congenital lesions of different, often the disease occurs at 4-5 months of fetal development, while the vulgar ichthyosis manifests itself after the first year of life. X-linked ichthyosis is also divided into several forms which vary in severity and clinical manifestations characteristic.

Fruit Harlequin   or ichthyosis fetus   one of the congenital form of the disease, development occurs in the embryonic period. The clinical picture of the disease is fully formed before birth.

Symptoms in newborns fetal ichthyosis is to complete the defeat of the skin. The skin is dry, thickened, horny shell covering consists of horny skin flaps with a gray-black color. Palms and soles also struck diffuse keratoderma . Ears, mouth and nose deformed, ugly limbs, eyelids, usually vyvorocheny. There may be other associated malformations of the fetus. Most children with ichthyosis fetus born dead. But the born alive die after birth, surviving children with such disabilities unit.

Ichthyosis epidermilogichesky   - Congenital form of the disease manifests itself immediately after birth. Characteristic symptoms of ichthyosis are expressed as " fumed fetus "- The fruit is covered with a film after the rejection, where the baby's skin looks scalded. There is a bright red color of the skin, the epidermis peeled off vast areas to form blisters and erosions. Actinic skin occurs at 3-4 year of life, with the horny layers of scallops have a concentric arrangement, layers thick, brown. "Colloidal fruit" is also observed in other forms of ichthyosis - lamellar ichthyosis . Dry ichthyosiform erythroderma .

There are several forms of ichthyosis, and which are extremely rare. Ichthyosis sided   It is characterized by one-sided localization of lesions. Affected half of the face, torso and limbs on the right or left side. As clinical signs of this form of distinction as one of the options ichthyosiform erythroderma. Ichthyosis echinated   - Very rare. At birth, manifested severe erythema Which later weakened and formed Linear verrucous areas with horny layers. Actinic and diffuse peeling resemble needle urchin.

Diagnosis ichthyosis

Initial diagnosis is based on the clinical picture of the disease. And for further diagnosis appointed further research.

Since it is possible, and this form of the disease as a Acquired ichthyosis That, in fact, is only able to provoke a Ichthyosiform other diseases, diagnosis of ichthyosis hereditary type of research carried out at the level of genes, cells, tissues. The basis of most forms of ichthyosis is violation of gene expression That encode forms keratin . Therefore, various disturbances and lead to various forms of the disease.

Since the diagnosis is found, for example, failure transglutaminase keratinotsidov   which is typical for lamellar ichthyosis . There has been rapid progress keratinotsidov to the skin surface. X-linked ichthyosis   diagnosed in the presence of strengthening ties of cells - Retention hyperkeratosis . It is characteristic and increase water loss through the skin. Violations keratinotsidov differentiation and development of defective keratin provoke blistering and thickening of the skin that diagnoses epidermolitichesky ichthyosis .

Complications ichthyosis

With age, the course of the disease is facilitated, unlike other diseases that are exacerbated, leading to complications. Complications ichthyosis often lie in concomitant diseases or birth defects. Often there is a complication such as a atopy   - increased allergic sensitivity . There is also lack of function of the endocrine system, and immune deficiencies ( immunodeficiencies ).

X-linked ichthyosis in its development can be followed underdevelopment, and pathology of genitals . There is a complication like hypogonadism , Sexual organs and secondary sexual characteristics do not develop due to the decrease in the secretion of hormones. Often, there are congenital conditions like cryptorchism   or monorchism , The absence of one, and sometimes both testicles in the scrotum.

The treatment of ichthyosis

In the treatment of ichthyosis is used complex therapy, mainly symptomatic treatment of ichthyosis. The main tool in the overall treatment Vitamin A   combined with a fortifying agent. Drugs with a high content of vitamin A And its analogs - retinoids Appointed cycles several times a year. Acceptance of these drugs is recommended 2-3 month course. It recommended constant surveillance while taking retinoids for early discontinuation in case of toxic effects on the body.

The treatment of ichthyosis requires moisture keratinized areas epidermal water loss through the skin is very significant and need constant rehydration. For this prescribed frequent baths, they may be salt, soda, starch, sulfide and others. Recommended treatment and the use of silt and peat mud.

From the exterior of medicines are appointed keratolytic agents   the content glycerol . propylene glycol   and lactic acid . These drugs are used without bandages. With occlusive dressing is recommended to use preparations containing salicylic acid , and urea .

In addition to medicines is assigned a number of physiotherapy: heliotherapy . thalassotherapy . rePUVA therapy . UV-rays .