tetracycline Update : Darier's disease (Darier-White's disease) is a keratinization disorder characterized by the development of keratinized papules in seborrheic areas and nail abnormalities. The MoyMinZdrav system allows you to identify a range of diseases and pathological conditions, the likelihood of which is higher with a certain set of symptoms in people of different sex and age. The MoyMinZdrav system does not replace a doctor and should only be used as an auxiliary tool in the work of medical professionals.

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Darier's disease (follicular vegetative dyskeratosis) is a hereditary disease transmitted in an autosomal dominant manner with varying degrees of phenotypic manifestation of a defective gene. The basis of clinical manifestations is pathological keratinization of epidermal cells (dyskeratosis). The primary element is small scaly macules or spherical papules, covered with crusts, merging into plaques. Dermatosis is localized on the face, chest, back, in the interscapular region, on the scalp. Possible secondary infection. There is no gender division. Treatment is symptomatic. The prognosis for life is favorable.

In massive osteolysis (Gorham's disease), progressive bone loss occurs, associated with hemangiomatosis or multiple lymphangiectasias. Patients are encouraged to use the MyMinHealth System to identify life-threatening conditions, as well as to select a doctor of a particular specialty for an initial consultation.

Causes of Darier's disease Classification of Darier's disease Symptoms of Darier's disease Diagnosis and treatment of Darier's disease Prices for treatment. Darier's disease is a rare hereditary dermatosis caused by an abnormal mutation of a part of DNA responsible for the synthesis of a protein that binds epidermal cells, resulting in dyskeratosis. This hereditary disease can be traced in several generations of the family, it manifests itself in the twentieth year of life. The pathology was first described in 1889 by the Frenchcue dermatologist J. Darier.

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It was a treatise on psorospermia, special microorganisms - lumps of protoplasm, which the researcher regarded as the root cause of the disease. Modern dermatologists are well aware that Darier's disease is a genetic disease that occurs due to a defect in the hereditary cell apparatus, affecting both women and men. The relevance of the disease is associated with the hereditary nature of the process, which requires an attentive and responsible attitude of parents towards their children, who are already growing next to them or are just planning to be born. There is no information on the prevalence of the disease.

Classic follicular dyskeratosis is a seborrheic form of the disease that occurs in 90% of cases. Localized (linear or zosteriform) dyskeratosis, an abortive form of the disease - linear foci of dyskeratosis are localized exclusively in limited and non-standard areas of the skin. Hypertrophic (hyperkeratotic) dyskeratosis or an isolated form (warty dyskeratoma) - the primary element is dyskeratosis plaques with cracks and warty growths on the surface. Vesicular-bullous dyskeratosis - characterized by polymorphism of the rash: nodules, vesicles at different stages of development.

 

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Frequently Asked Questions

Darier-White disease is a relatively rare hereditary disease. It is inherited in an autosomal dominant manner. Both sexes are affected with equal frequency. The mechanism of development of this disease is still unclear.

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The first signs of the disease usually appear in childhood or adolescence, gradually progressing. The child's condition deteriorates sharply after exposure to sunlight. The disease is manifested by the rash of a large number of bubbles. At the first moment, the color of the emerging bubbles is no different from the color of the skin.

Such changes in the skin also affect the folds, which distinguishes this disease from many of tetracycline listed above. The formation of crusts on the skin is accompanied by their weeping. First of all, the rash occurs on the skin of the face, scalp, behind the auricles, in the sternum and between the shoulder blades, in the folds.

Subsequently, they acquire a grayish-brownish color, covered with small crusts. Initially, the crusts are located in isolation from each other. As the disease progresses, the crusts begin to merge with each other, forming foci of a rather large size..