Folkericular dyskeratosis( Darjeel White disease)

Darya-White disease is a relatively rare hereditary disease. It is inherited by an autosomal dominant type. Both sexes are affected with the same frequency. The mechanism of the development of this disease is still unclear.

The first signs of the disease appear usually in childhood or adolescence, gradually progressing. The child's condition deteriorates sharply after being exposed to the sun's rays. The disease is manifested by the precipitation of a large number of vesicles. At first, the color of the emerging bubbles is no different from the color of the skin. Subsequently, they acquire a greyish-brownish color, are covered with small crusts. At first, the crusts are isolated from each other. As the disease progresses, the crusts begin to merge, forming foci of fairly large size. Such skin changes also affect the folds, than this disease differs from many listed above. The formation of crusts on the skin is accompanied by their wetting. First of all, the rash occurs on the skin of the face, the scalp, behind the auricles, in the sternum and between the shoulder blades, in the folds. In severe cases, the entire skin can be affected. Sometimes the mucous membrane of the oral cavity is involved in the process.

Darya-White disease can occur in the form of a bullous form( with the appearance on the skin of large enough blisters), only single foci can appear. Isolated variant of the Darje-White disease is the warty discretoma. With this variant of the disease on the skin of the rear of the hands and feet, the emerging vesicles look like warts. In this case, such people often have nail changes, there may be point keratoses on the palms and soles. Some may note a decrease in intellectual abilities. Quite often, this skin disease is complicated by infection, which, apparently, is associated with impairment of the immune system.

Treatment of is carried out with vitamin A in elevated doses, as well as with tigazone. When the skin is infected, various antibacterial agents are used.