Most people are exact copies of their parents. Some get blue eyes from dad, others get mom's gorgeous hair. Of course, the opposite also happens. However, we all grow up as the most interesting result of the interweaving of the genes of our close relatives. Very often, along with big eyes, curly hair and long legs, parents give their children a set of various diseases. They do it 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
Dariaer's disease refers to hereditary dermatosis, manifested by rashes in the form of brown or brown nodules. They have a conical shape and are covered with dense crusts. Nodules localized on the folds of the body merge together and form weeping wounds. Depending on the location of the rashes and their appearance, Darier's disease is divided intofour shapes:
- Localized. It is characterized by a linear arrangement of lesions that cover only limited areas of the body.
- Classic. The rash is predominantly localized on the scalp, chest and behind the ears.
- Isolated. Together with standard nodules, multifaceted papules appear that look like warts. As a rule, they are located 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 side of the neck, in the area of large skin folds.
Primary foci of papules are diagnosed in patients aged eight to fifteen years. The disease affects adults extremely rarely.
Causes of illness
The main cause of this disease is the presence of an abnormal gene, and the main risk factor is a hereditary 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 decreases. The consequence of these pathological processes is a violation of the mechanism of keratinization of the skin.
If mom or dad are carriers of an abnormal gene, in 50% of cases a child is born with a diagnosis of Darier's disease. However, if an absolutely he althy baby is born to sick parents, then this pathology will not bother his future children. The disease is not transmitted by airborne droplets or sexual contact.
Clinicalpicture
The pathological process begins with the appearance of follicular flat nodules, which are otherwise called papules. Their size can vary from 1 to 3 mm. The papules are covered with a hard brown crust. Under it are recesses in the form of a funnel. Nodules are localized in the mouths of the hair follicles, which sometimes leads to the development of another disease - folliculitis. Therefore, a differential diagnosis must be made before starting treatment.
Rashes are located symmetrically, capturing the subclavian region and flexion surfaces of the joints. On the scalp, papules resemble warty plaques with scaly crusts. After their removal, foci of baldness appear in the lesions. As the disease progresses, new rashes appear, and old ones merge into a single spot.
Medical examination
Diagnosis of the disease is based on 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 disease, a histological analysis of a skin biopsy is prescribed. The differential diagnosis is with familial pemphigus, papillomatosis, and seborrheic dermatitis.
Principles of Therapy
Treatment for this disease is usually done 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. Everyonepatients, without exception, are prescribed vitamin A orally or in the form of intramuscular injections. 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.
A positive result in Darya's disease is given by pearl baths in combination with applications of keratolic ointment. In the presence of extensive lesions, surgical intervention or electrocoagulation is indicated. When Darier's disease is accompanied by 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 whose family has already been diagnosed with Darier's disease.
The treatments described above usually show good results. Many patients manage to achieve stable remission for several years. However, a full recovery should not be expected.
