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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.
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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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The classic form of the disease is manifested by a rash of follicular papules covered with scabs (crusts) on areas of the skin that are typical for the localization of the seborrheic process: forehead, nasolabial folds, temples, neck, décolleté, interscapular space. The scalp is also affected. When the crusts are removed, small erosions form, which become inflamed due to the addition of a secondary infection, causing pain, an unpleasant odor, and general intoxication of the body.
Antibiotic Treatment
With a localized or abortive form of the disease, rashes are located linearly only on a limited area of tetracycline pills: the primary element is papule, the secondary is pigmentation and erosion. The isolated form is characterized by a combination of nodules and polygonal papules that resemble warts and tend to coalesce into plaques. The process is localized on the back of the hands and feet. In this case, the palms and soles are affected by diffuse punctate keratosis.
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With a vesicular-bullous variety of Darier's disease, vesicles with transparent contents join the nodules. The rash is localized in large folds of the skin and mucous membranes. If the process affects exclusively the mucous membranes, they speak of leukoplakia, which requires special attention.
Cataracts, myopia, nystagmus, strabismus, diseases of the endocrine glands, epileptic seizures, dementia, bone tissue cysts occur as a background pathology of follicular vegetative dyskeratosis. Dermatosis has an undulating course with long remissions and exacerbations. The provocative moment is most often UVI.
Individual Therapy
Treatment is symptomatic, long-term and complex. First of all, it is necessary to exclude factors that can provoke an exacerbation or relapse of the disease: UVI, hypothermia, high humidity, sweating. Next - get rid of the secondary infection, if any, with the help of antibiotics (amoxicillin + clavulanic acid), antifungal drugs (ketoconazole). Aniline dyes, aerosols based on antibiotics and hormones are used locally. With a pronounced inflammatory process with an exudative component, glucocorticoids are used orally.
Couples Therapy
At the same time, foci of chronic infection are sanitized and concomitant somatic pathology is treated. The main attention is paid to the correction of keratinization, using vitamin A in large doses, vitamin E. Salicylic ointments (2% -5%), Unna cream with retinol, dimethyl sulfoxide are prescribed externally. Oxygen therapy, oxygen and sea baths are shown. PUVA therapy recommended. Elements of large sizes are removed after consultation with a dermatologist and dermatocosmetologist. The prognosis is favorable. Under the influence of tetracycline therapy or spontaneously, in some cases, long-term and persistent remissions are possible, but recovery is impossible.
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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..