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December 29, 2016 00:08

Vegetating follicular dyskeratosis |Symptoms and treatment of vegetating follicular dyskeratosis

vegetating follicular dyskeratosis (syn Darier's disease.) - Dermatosis, inherited in an autosomal dominant manner.Three clinical variants are described: classical;localized (linear or zosteriformnaya);warty diskeratoma.

Symptoms Darya

Clinical manifestations of the disease occur in children, the process takes a chronic course with a tendency to progression.The rash is usually localized on the seborrheic areas of the breast skin, back, scalp, behind the ear, but can spread to the skin of the extremities, face, hitting the oral mucosa.Characterized keratotic follicular papules normal skin color or yellowish-brown, covered with small crusts, verrucous papules are also found with symptoms of soak.There may be vesicular-bullous rash, changes in the nail plate, described palmar-plantar keratosis point, the combination of a bone cyst.On the back of hands are frequently found lesions that resemble common warts, most likely corresponding to the classical picture akrokeratoza Hopf.Often dermatosis complica

ted by secondary infection.

Pathology vegetating follicular dyskeratosis

Darier's disease is characterized nadbazalnym acantholysis to form slots containing acantholytic cells and proliferation of dermal papilla, vystupayushih into the cavity of the bubble.Dyskeratosis is detected more often in the areas of education gaps in the form of "round cells in the granular and grains -. In the horny layers of the Round calf are epithelial cells rounded, not associated with surrounding cells with basophilic homogeneous cytoplasm, pyknotic nucleus and bright rim on the periphery of the grains. -. homogeneous eosinophilic education with subtle nuclei or without them in the epidermis marked giperortokeratoz with the formation of horny plugs in the mouths of hair follicles, acanthosis, papillomatosis in the dermis -. perivascular lymphohistiocytic infiltrate with isolated eosinophilic granulocytes

According to electron microscopic studies, round cells.predetavlyayut a large cage, around the core of which is a broad band of vacuoles, and on the periphery of the cells -. organelles similar structures have and acantholytic cells vacuolation cytoplasm amplified as you move cells to the surface of the epidermis, in the granular layer tonofilaments and related pellets eleidin pushed to.cell membrane, lamellar granules predominate among organelles.Grains contain diffusely located in the cytoplasm grained and fine fiber structure, the core is absent.In the upper layers of the epidermis are lysed epithelial cells probably represent the final stage of the existence of round cells.In acantholysis zone are atypical desmosomes.in which the middle layer is absent or is enlightened, uneven contrast, as if eaten.

Histogenesis vegetating follicular dyskeratosis

According to electron microscopic studies, the main processes taking place and the epidermis, characterized by vacuolization of individual epithelial cells, progressing as you move cells to the surface of the epidermis and condensation in them tonofilaments, last are connected with major keratogialinovymi granules, visible alreadyin the thorny layer.I. B. Caulfield described the process of premature keratinization.Previously it was assumed that the grain - the final stage of differentiation of round cells, however, as the grains do not contain keratin, probably they are formed independently of the round Thelen.The basis acantholysis with Darier's disease is considered defective formation of desmosomes, loss of contact with tonofilaments desmosomes and intercellular cementing substance defect.

in the pathogenesis of the disease are important and Darya Drut factors. Reducing tensions cellular immunity, the activity of certain enzymes (NADP-dependent and T-6-FDP ,. participating in the process of keratinization is assumed the important role of vitamin A deficiency, indirectproof of which can be successfully treated the disease aromatic retinoids and vitamin A.