Most people are replicas of their parents. One gets blue eyes from dad, the other - chic mother's hair. Of course, it happens and vice versa. However, we all grow up as an interesting result of the interweaving of the genes of our close relatives. Very often, together with big eyes, curly hair and long legs, parents give their children a set of various diseases. They do this not on purpose, even without their own consent. Pathology may not manifest itself throughout life, but more often the situation is different. A person becomes a hostage to family ailments and ailments. One of these “gifts” is Darier’s disease. What it is? Why does this pathology develop?
general information
Daria’s disease refers to hereditary dermatosis, manifested by rashes in the form of nodules of brown or brown color. They have a conical shape and are covered with dense crusts. Nodules localized on the folds of the body merge together and form wet wounds. Depending on the location of the rashes and their appearance, Darier’s disease is divided into four forms:
- Localized. It is characterized by a linear arrangement of foci that cover only limited areas of the body.
- Classical Rashes are mainly localized on the scalp, chest and behind the ears.
- Isolated. Along with standard nodules, multifaceted papules appear that look like warts. As a rule, they are on the back of the hands and feet.
- Vesicular-bullous. This form of the disease is very rare. It is characterized by numerous rashes with a transparent secret. They appear on the lateral surface of the neck, in the area of ​​large skin folds.
Primary foci of papules are diagnosed in patients aged eight to fifteen years. Adult illness is extremely rare.
The causes of the disease
The main cause of this disease is the presence of an abnormal gene, and the main risk factor is the inherited predisposition. As a result, vitamin A is not fully absorbed by the body, the activity of certain enzymes and the zinc content in the epidermis are reduced. The consequence of these pathological processes is a violation of the keratinization mechanism of the skin.
If mom or dad are carriers of the abnormal gene, in 50% of cases a child is born with a diagnosis of Darier’s disease. However, if an absolutely healthy baby is born in sick parents, then this pathology will not bother his future children. The disease is not transmitted by airborne droplets or sexually.
Clinical picture
The pathological process begins with the appearance of follicular flat nodules, which are also called papules. Their size can vary from 1 to 3 mm. Papules are covered with a hard crust of brown color. Underneath are recesses in the shape of a funnel. Nodules are localized in the mouths of the hair follicles, which sometimes leads to the development of another disease - folliculitis. Therefore, before starting treatment, it is necessary to conduct a differential diagnosis.
Rashes are located symmetrically, capturing the subclavian region and the flexion surfaces of the joints. On the scalp, papules resemble warty plaques with flaky crusts. After their removal, lesions appear in the lesion sites. As the disease progresses, new rashes appear, and the old ones merge into a single spot.
Medical examination
Diagnosis of the disease is carried out on the basis of the clinical picture and a complete history of the patient. During the examination, the doctor examines the symptoms of pathology, previous cases of rashes among close relatives. To confirm the ailment, a histological analysis of a skin biopsy is prescribed. The differential diagnosis is carried out with familial pemphigus, papillomatosis and seborrheic dermatitis.
Principles of Therapy
Treatment with this ailment, as a rule, is carried out on an outpatient basis. With an exacerbation of the pathological process or the appearance of extensive lesions, the patient requires hospitalization in a dermatological hospital.
The patient is shown symptomatic therapy. All patients, without exception, are prescribed vitamin A orally or intramuscularly. The dosage of the drug is calculated individually, taking into account the age of the patient. The course of treatment is usually 3 months, then take a break and repeat the therapy.
Pearl baths in combination with keratolic ointment applications give a positive result for Darier’s disease. In the presence of extensive lesions, surgical intervention or electrocoagulation is indicated. When Darier's disease is accompanied by the addition of a bacterial infection, antibiotics are prescribed.
Forecast and Prevention
This disease is transmitted exclusively by inheritance. That is why, as a primary prevention, experts recommend a consultation with a geneticist for couples planning to conceive a child in the near future. This is especially true for future parents who have already been diagnosed with Darier's disease in the family.
The treatment described above usually shows good results. Many patients manage to achieve persistent remission for several years. However, a complete recovery is not worth the wait.