In relation to the class of skin diseases, ichthyosymorphic erythroderma is a disease that affects the upper layer of the epidermis with the formation of inflammation sites that are covered with blisters and many small dry scales. This is a congenital form of ichthyosis, which has a bullous form and is difficult to cure. To date, there is no precise data on the prevalence of this disease.
Features of the disease
Equally manifested in men and women, their ichthyosiform erythrodermia affects the upper layers of the skin of the body, manifesting itself as constant peeling, the appearance of small dry scales and blisters. Transmitted as a hereditary factor, the disease begins as a result of gene mutation. An important role, according to the latest studies of the disease under consideration, is an increase in the level of n-alkanes. The mutation occurs in the gene that encodes the formation of epidermal trans-glutaminase.
The most commonly affected areas are axillary cavities, areas of large joints, inguinal folds - in these areas lesions have a pronounced concentric shape. Inflammatory processes in the upper layer of the epidermis are accompanied by hyperkeratosis, with a different degree of pronounced acanthosis. Also, there may be an accumulation of horny plugs, especially at the mouth of the hair follicles, which hinders the growth and nourishment of the hair. There is a deterioration in the patient's hair.
Congenital bullous ichthyosiform erythroderma( photos)
Classification of ichthyosiform erythroderma
Today, there are many different forms of erythroderma that have similar symptoms and manifestations. To clarify the diagnosis should be a number of additional laboratory studies that will make the most optimal treatment regimen.
This disease is classified according to the severity of its course:
- of medium severity , in which only some areas are affected( mainly the areas of axillary hollows and inguinal folds);
- is a severe degree of lesion, which is characterized by significant areas of skin damage.
There are also a number of syndromes, including ichthyosiform erythroderma:
- , the Netherton syndrome;
- syndrome Conradi-Hyunermann;
- Sjogren-Larsson syndrome;
- syndrome of accumulation of neutral fats;
- Tau syndrome.
Causes of
In the opinion of some dermatologists, the disease under consideration can arise due not only to a hereditary gene mutation, but also to the occurrence of disorders in the synthesis of tonofilament, inability to remove platelet granules from epitheliocytes. Inheritance of this disease occurs in an autosomal dominant type.
Symptoms of
The manifestations of ichthyosiform erythroderma are very typical for the whole variety of forms of ichthyosis, in which the upper layer of the epidermis is affected and symptoms such as:
- dryness of the skin, a feeling of tightness;
- on the surface of the skin gradually formed a large number of dry scales, which can have from light gray to dark brown hue;
- the skin in the lesions acquires a bright color, the hair on it becomes less and less due to the constant clogging of the mouths of the hair follicles with granules of dead skin;
- is a frequent form of the disease - a congenital kind of disease in which an infant is already born in a damaged skin. Most often in this case, the affected areas of the face, trunk;On a body linearly located eruptions of a verrux character. Plantar and palmar keratosis can also be observed.
Since it is n-alkanes that are responsible for the solidification of the intercellular substance, in the case of disturbances in their production and accumulation, the brittleness, excessive hardness of the surface( horn) layer of the epidermis increases.
Diagnostics
- For the preliminary diagnosis, an experienced dermatologist will have enough time to examine the patient;However, due to the large number of ichthyosis species, it is necessary to analyze such indicators as the presence of dilated vessels in the dermis, the degree of atrophy of the hair follicles and sebaceous ducts, whether there are perivascular and perifollicular inflammatory infiltrates in the surface layer of the skin. These data are specific for their ichthyosiform erythroderma.
- Also used are diagnostic methods, such as antenatal methods. In the presence of clots of tonofilament, as well as the absence of complexes of tonofibrils, this disease can be diagnosed: in the skin of healthy people, they are absent in the cellular granular layer. Analyzes of the amniotic fluid also make it possible to diagnose during pregnancy: the presence of epithelial cells having pycnotic nuclei and containing tonofilament aggregates also indicate a high probability of ichthyosiform erythroderma development in the fetus.
Treatment of
Complete recovery of the disease under consideration is impossible today. However, a comprehensive approach to treatment allows in a short time to significantly improve the overall skin condition, eliminate the active formation of dry scales on its surface.
Therapeutic way of
To improve the skin condition, a dermatologist can prescribe drugs with a high content of vitamin A, which has a positive effect on the general condition of the skin and gives the skin elasticity and firmness.
Vitaminotherapy helps to improve the level of immunity, which is also important in the development of ichthyosiform erythroderma. Complex vitamins should be prescribed by a dermatologist, they should contain vitamin A.
By medicamentous method
The use of reytoids can reduce the process of skin cell rejection, which stops peeling, and moisturizers, especially after hygienic procedures, will restore its softness, elasticity and elasticity.
Among other medicines in the treatment of the disease in question, particular attention is given to Acitretin, which is taken orally in an amount of 10-35 mg / day. Duration of treatment is 4 weeks, then a break should follow, then the treatment course is repeated. To the keratolytic agents helping to restore the skin condition and reduce the symptoms of the disease, the following should be attributed:
- urea, ointment 2-11%, applied topically;
- hepatoprotectors in combination with fortifying agents;
- ointment Lactic acid( 3-6%), also applied topically until the appearance of the improvement.
The evaluation of the effectiveness of the treatment is based on the analysis of the skin condition, the reduction of clinical manifestations of the disease.
Epidrodermia of Wilson Brok( photo)
Prevention of
- As this type of dermatological disease is inherited, couples who plan a pregnancy, if they have or their closest relatives have a history of erythrodermia of a different nature, you should undergo a general examination and consult a dermatologist.
- Also to maintain improved after the treatment, you should regularly carry out hygiene procedures using soft exfoliating drugs, special gels for dry skin and ointments based on urea.
Forecast of
At the birth of a child with congenital erythroderma, treatment with retinoids should be immediately applied, thus saving his life. When diagnosing the disease in adulthood, to improve the quality of life, all the recommendations of a dermatologist should be followed, as well as the prescribed treatment.