Epidermolysis Bullous Congenital Simple

Epidermolysis Bullous Congenital Simple: Description, Causes and Treatment

Epidermolysis bullosa congenita simplex (EBVP) is a genetic disorder that causes blisters to form on the skin and mucous membranes of newborns and infants. The disease belongs to the group of epidermolysis, which is characterized by increased susceptibility of the skin to injury and friction.

Causes and symptoms

EBVP is caused by a disruption of the genes responsible for the synthesis of keratin, a protein that provides strength and elasticity to the skin. This causes the skin to become very fragile and easily damaged, even with light rubbing.

The main symptom of EBVP is blisters on the skin and mucous membranes. The blisters can be small or large, filled with fluid or blood, and can occur in any part of the body. The skin where blisters form can be very thin and easily injured.

Other symptoms of EBVP may include:

1. formation of scars on the skin;
2. increased susceptibility to infections;
3. increase in the size of fingernails and toenails;
4. changes in skin color and texture;
5. problems with teeth and gums.

Treatment

EBVP does not have a complete cure because it is a genetic disorder. Treatment is aimed at relieving symptoms and preventing complications. The main treatment measures may be:

1. prevention of injury to the skin and mucous membranes;
2. use of soft and non-slip surfaces for sleeping and resting;
3. avoiding contact with irritating substances;
4. treatment of infections if they occur;
5. using special dressings to protect the skin and reduce the risk of blistering;
6. taking vitamins and nutrients to maintain healthy skin and strengthen the immune system.

In some cases, surgery may be required to correct bone deformities or other complications.

In conclusion, EBVP is a rare but serious condition that requires constant medical monitoring and care. Early detection and timely treatment can help reduce the risk of complications and improve patients' quality of life.

Epidermolysis is a skin disease characterized by the formation of blisters on its surface. A characteristic symptom is the appearance of blisters at the slightest damage to the skin. An important place is occupied by the hereditary nature of the disease, in which genetic factors play an important role. The causative agent of the disease was not detected, but its histological sign was identified. Currently, the concept of “congenital epidermolysis” has been introduced into clinical practice - a Latinized version of the term “epidermolase”, which was used in Russia in previous years. Thus, the term implies both the presence of bubbles and the reason for their appearance.