May
18
23:01
Genetic diseases

Darier's disease

Darier disease

Darier's disease (psorospermoz, follicular dyskeratosis, vegetating keratosis) - a chronic genetic disease of the skin, which is characterized by the appearance of the cone-shaped brownish or yellowish-brown nodules specific to five millimeters in diameter.These nodules for the most part associated with hair follicles and their surface is covered with thick crusts of gray-yellow

Symptoms

- presence on the skin of multiple dense papules (knots) often spherical shape with a diameter of two to five millimeters, with a rough scaly surface.The color of these formations varies from normal skin color to gray - brown.Often papules coalesce, thereby forming oozing lesions, found in skin folds
- to the nail plate appears white and red longitudinal stripes, in some cases, there is their longitudinal splitting
- Perhaps the mucous membranes of the larynx, pharynx, oral cavity, esophagus,rectum, vulva and vagina
- may develop subungual hyperkeratosis

Causes

The main cause of this disease is considered to occur in both sexes, the defective gene (fifty percent of the children passed by hereditary line).In healthy children with a parent born very healthy offspring.Darier's disease is transmitted by the type of vertical inheritance (if not impaired reproductive function).
main risk factor is genetic predisposition

main forms of disease Dare

- Localized form: asymmetrically located on localized areas of elongated lesions
- Classic follicular dyskeratosis: symmetrically arranged nodular lesions on the scalp, face, behind the ears, betweenvanes in the sternum and large folds
- isolated form: in addition to the typical knots from the back of the feet and hands appear warty kind of multifaceted papules that form plaques at the confluence
- Vezikullezno bullous form: in addition to papules formed bubbles filled with clear content.Such foci appear on the sides of the neck and in the large folds.On the soles and palms may develop a point keratosis (the appearance of keratinous areas)

In most cases diagnosed solely on clinical grounds, but in case of doubts about the established diagnosis is performed histological examination of affected skin

Treatment

- vitamin A supplementation
- Reception keratolytic drugs
- In order to reduce the risk of side effects and increase the effectiveness of treatment of vitamin A, is assigned to receive hepatic and vitamins
- Laser
- Cryotherapy
- Diathermocoagulation
- Physiotherapy (radon and sulphide bath)
Treatment of this disease is usually limited to ambulatory and only in cases of acute dermatological patients are sent to hospital.

also need to know that the untimely treatment of Darier's disease may develop serious complications:
- Mental retardation
- viral or bacterial infection of the skin
- Epilepsy
- Various skeletal anomalies
- endocrinopathy
- Ocular
prognosis for life is quite favorableand to avoid possible exacerbations should be possible to avoid UV radiation and prolonged exposure to the sun.

service physician recruitment is relevant only for the citizens of the Russian Federation

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