Vegetative follicular dyskeratosis: causes, symptoms, diagnosis, treatment

Growing follicular dyskeratosis (blue Darya disease) is a dermatosis inherited in an autosomal dominant type. Three clinical varieties are described: classical; localized (linear or zosteriformnaya); warty discorate. Clinical manifestations of the disease occur in childhood, the process takes a chronic course with a tendency to progress. Eruptions are usually localized on the seborrheal areas of the skin of the chest, back, scalp, and behind-the-ear area, but can spread to the skin of the limbs, face, affect the mucous membrane of the oral cavity. Characteristic keratotic follicular papules of normal skin color or yellowish-brown, covered with small crusts, there are also verucous papules with the phenomena of wetness. There may be vesicular-bullous eruptions, changes in nail plates, palmar-plantar spot-like keratoses, a combination with cysts of bones are described. On the back of the hands, rashes often appear that resemble common warts, most likely corresponding to the classical picture of acrokeratosis of Hopf. Often, dermatosis is complicated by a secondary infection.

Pathomorphology of vegetative follicular dyskeratosis. Darya's disease is characterized by a sub-basal acantholysis with the formation of cracks containing acantholytic cells, and the growth of papillae of the dermis protruding into the cavity of the bladder. Dyskeratosis occurs more often in the areas of formation of gaps in the form of "round corpuscles in granular and granules in the stratum corneum, round bodies are rounded epitheliocytes that are not associated with surrounding cells, having a basophilic homogeneous cytoplasm, pycnotic nucleus and a bright rim around the periphery, homogeneous eosinophil formations with hardly noticeable nuclei or without them.In the epidermis hyperkerthokeratosis is noted with the formation of corneous plugs in the mouths of the hair follicles, acanthosis, papillomatosis.In the dermis - perivascular mfogistiotsitarny infiltrate with isolated eosinophilic granulocytes.

According to electron microscopic data, round corpuscles are represented by large cells around the nucleus of which there is a wide band of vacuoles, and around the periphery of the cell are organelles. Acantholytic cells also have a similar structure. Vacuolization of the cytoplasm increases as the cells move to the surface of the epidermis, in the granular layer, the tonofilament and the keratogialin granules bound to them are pushed back to the cell wall, among the organelles the lamellar granules predominate. Grains contain fine-grained and fine-fibrous structures diffusely located in the cytoplasm, the core is absent. In the upper layers of the epidermis, lysed epithelial cells are found, probably representing the final stage of the existence of round bodies. Atypical desmosomes are found in the acantholysis zone. In which the middle layer is absent or enlightened, unevenly contrasted, as it were eaten.

Histogenesis of vegetative follicular dyskeratosis. According to the electron microscopic data, the main processes taking place and the epidermis are characterized by vacuolization of individual epitheliocytes progressing as cells move to the surface of the epidermis and condensation of the tonofilament in them. The latter are connected with large keratogialin granules that are already visible in the spiny layer. IB Caulfield called this process a premature keratinization. Earlier it was assumed that the grains are the final stage of differentiation of round bodies, however, since the grains do not contain keratin, they probably are formed independently of the round calves. The basis of acantholysis in Darya's disease is the formation of defective desmosomes, the loss of contacts of the tonofilament with desmosomes, and the defect of the intercellular cementing substance.

In the pathogenesis of Darya's disease, factors such as the decrease in the intensity of cellular immunity, the activity of certain enzymes (NADP-dependent and G-6-PDPA, involved in the keratinization process, have an important role in vitamin A deficiency, indirect evidence of which can be successful treatment diseases with aromatic retinoids and vitamin A.

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