With regard to skin diseases, Dereh's disease as a kind of congenital ichthyosis can be characterized as one of the varieties of dyskeratosis, accompanied by a violation of the process of keratinization of the upper layer of the epidermis. The absence of data and the prevalence of the disease do not allow us to judge its epidemiology, but manifestations and symptoms can occur quite often, since there are several stages of the development of the disease and they all differ in their manifestations.
Today we will tell you how follicular dyskeratosis manifests itself in the fetus, newborns, in children, in adults, what are the causes of congenital ichthyosis and how to treat it properly.
Features of the disease
Manifestations in women and men do not differ;the main symptom of the disease is the appearance of seals on the skin, which arise due to the presence of a defective gene. Darya's disease is inherited, as the gene mutation can be transmitted to both men and women. In this case, the autosomal dominance of the mutated gene is observed.
The basis of Darya's disease is a combination of the following factors:
- structural changes in the composition of desmosomes;
- development of disturbances in the formation of complexes of to-no-filaments-desmosomes;
- occurrence of a defect in the composition and state of intercellular cement;
- acceleration of the process of vacuolization of individual epithelial cells with active condensation in them of a large number of tonofilaments, which leads to the formation of cells of two types - grains and round bodies.
In this case, an increase in the activity of enzymes that take part in the keratinization of the epidermis, as well as reduced cellular immunity by reducing the content of zinc in the skin cells.
Daryer's Disease( photo)
Today there are four main clinical forms of Darjean disease:
- classic follicular;
- localized( zosteriform or linear);
- is vesicular-bullous.
The development of the disease is mainly observed in adolescence. The nature of Darya's disease is chronic with gradual progression in the summer: the skin becomes more susceptible to sunlight( UV rays).
The main causes of the onset of the Darya disease are gene changes that have the property of being inherited by both men and women. Inheritance is autosomal dominant.
The most frequent manifestations of Daria's disease are changes in the skin that gradually increase in thickness become thicker and coarser. Probably the manifestation of peeling, with various forms of the disease, the peeling has a different intensity.
Also, the external manifestations of the activation of Darya's disease have the following symptoms:
- the appearance of multiple dense papules with a follicular character, whose diameter is 0.3-0.6 cm. They have a globular shape with a papillomatous plane on the surface, which is covered with minute flakes;
- papules on the skin have a pronounced greyish-brown color, rarely - skin color;
- skin lesions are observed mainly in the face and scalp, in the chest area, behind the ears, in the area of large folds;
- at the confluence of individual papules arise verruzic plaques. Also, small bubbles can develop on the skin, often in the neck.
Changes in the condition of the nail plate can often be observed: longitudinal bands of white and red colors appear, the nails begin to separate.
Congenital ichthyosis in a child( photo)
Diagnosis of Darya disease
The clinical picture allows you to put a preliminary diagnosis, but to refine it and determine the method of treatment, a number of additional studies of the affected skin should be carried out. The laboratory method of investigation is histological examination.
The following treatment is considered, aimed at congenital ichthyosis in the form of Darya disease.
Complete cure of Darya's disease is not achieved, but modern methods of treatment can quickly and permanently eliminate the unpleasant manifestations of this pathological change and restore health to the skin.
About the treatment of the disease Daria will tell this video:
- The appearance of the keratinized areas on the skin can be treated with the use of preparations of vitamin A, which improves the skin condition and allows to reduce the manifestations of the disease. Vitamin A also improves the absorption of zinc from multivitamin preparations and food, which also positively affects the skin. Treatment is usually done on an outpatient basis.
- Also used is the use of pearl baths with keratolytic ointment in the form of appliqués.
By medicamentous method
In case of exacerbation of Darya's disease, the patient is usually hospitalized. During treatment, he is given oral intake of vitamin A, as well as his injections. The duration of treatment is selected individually.
- For particularly complex cases of skin lesions in Darya disease, surgical intervention or electrocoagulation may be prescribed.
- With parallel development of bacterial infection, antibacterial therapy should be used.
- Disease Because Darya's disease is transmitted only by inheritance, counseling can be recommended as a prophylaxis for a dermatologist in the presence of this disease in one of the relatives of couples who are planning a pregnancy.
- Regular hygiene procedures, drying the skin ointments and creams, the use of special means for showering and bathing - all these moments must be observed with a tendency to skin diseases.
About what complications can lead to the presence in the history of Darya's disease( follicular dyskeratosis disease), we will tell below.
Follicular dysyratosis( photo)
In the absence of necessary treatment, complications can arise in the form of exacerbation of external manifestations, the appearance of puffiness of the skin, deterioration of its condition with eruptions.
Treatment prescribed in a timely manner and carried out in a complex way, allows to obtain stable improvement of skin condition. However, a full recovery should not be expected.