How is dermatolysis bullosa treated?

Skin diseases are very common in life because the skin is the first barrier that isolates the human body from the outside world. Most bacteria and viruses invade the body through the skin, which greatly increases the probability of skin diseases. There are many types of skin diseases, and the treatments are complicated, which makes patients feel very painful. Let’s take a look at how to treat dermatolysis bullosa.

Epidermolysis bullosa is a blistering skin disease that does not leave scars. It is also a multi-gene inherited skin disease characterized by susceptibility of the skin and mucous membranes to mechanical damage and the formation of blisters; internal organs may also be affected. Clinically, the disease shows great variability.

Although the genetic basis has been elucidated, there is still no effective treatment. The most effective approaches are counseling and prevention. The treatment of this disease is mainly aimed at its secondary infection. The principle is to provide careful care, protect the local area, avoid trauma, friction, and heat, and prevent secondary infection. Living in a cool environment and avoiding high temperatures can be beneficial for some patients. Because the disease occurs at birth or in early infancy and is often caused by trauma, care for the child is difficult, such as avoiding traumatic activities.

1. Western medicine treatment

Vitamin E 100 mg, 3 times/day and sodium citrate 2 g, 3 times/day can be used. For severe patients, especially those with lethal disease, corticosteroids can be used. The starting dose for newborns is 140 mg/d, taken in several divided doses. Phenytoin sodium is a promising drug for the treatment of malnutrition-related epidermolysis bullosa. The drug can inhibit collagenase in the skin. The initial dose is 5 mg/(kg·d) in several divided doses, and the dose is gradually increased to 8 mg/(kg·d). After half a year, it is reduced to 6 mg/(kg·d). After one year, it is changed to the daily dose taken every other day.

Antibiotics may be used to prevent or control secondary infection. Severe patients, especially those with fatal types and severe anemia, often require blood transfusions or other supportive therapies.

2. Gene therapy

Epidermolysis bullosa simplex is often inherited in an autosomal dominant manner, making genetic therapy more difficult because expression of the mutant allele must be recessive. The current approach is to remove the patient's defective allele through homologous recombination technology. Although it is feasible to culture basal keratinocytes from patient skin biopsies, the frequency of homologous recombination is extremely low.