Butterfly skin: what is it, symptoms and causes

Under normal conditions, the skin is mainly made up of two cell layers: the epidermis (the upper layer) and the dermis (located below it), in turn, they are strongly linked by a large set of proteins that together are called the basement membrane. . In addition, multiple other proteins are responsible for joining the cells of each layer to each other.

In people with butterfly skin, some genes responsible for producing these proteins are defective, which means that this union is not as strong as it should be. Mutations can be inherited from one or both parents, or be spontaneous , that is, those that occur after birth and are often called butterfly skin in adults.

Therefore, we can say that the main risk factor for butterfly skin disease is having a family history of this disorder.

Although there are around 30 subtypes of bullous epidermolysis, this disease can be classified into three large groups, each presenting a series of different symptoms:

Simple blister epidermolysis

It is the most common form, around 70% of people have this type. In this type of butterfly skin, the break in the tissue is made in the most superficial layer of the skin, the epidermis . Symptoms are limited to blistering mainly on the hands and feet, but can occur all over the body. With good wound and pain management, people with this condition can lead relatively normal lives and may improve over time.

Dystrophic blister epidermolysis

It is the second most common form, affecting 25% of people with this condition. In this subgroup, the blisters appear in the deepest layer, the dermis , which upon healing will cause retractions in the joints, which can make movement difficult. Symptoms are more variable and include:

• Joint pain
• Fusion of fingers and toes
• Contraction of the mucosa inside the mouth
• Narrowing of the esophagus, which leads to difficulty in eating solid foods, and in more advanced stages, liquids.

People with this type of butterfly skin have been found to have an increased risk of developing squamous cell cancer (aggressive) before the age of 35.

Joint blister epidermolysis

It is the rarest type of butterfly skin and affects only 5% of people with this condition. In this type of epidermolysis bullosa, the problem occurs in the basement membrane , the layer that joins the epidermis with the dermis. Symptoms include:

• Marked skin damage.
• Blisters in the oral tract.
• Large number of blisters throughout the body.
• Blisters on the membranes of internal organs.
• Severe complications that can lead to death.

Currently there is no treatment to cure butterfly skin disease, but the treatment is aimed at minimizing the possible complications that wounds and blisters can cause in the patient and guarantee an improvement in the patient’s quality of life.

Constant patient care must be performed that includes:

• The blisters have to be pierced, drained and covered with all possible hygiene measures.
• A general bandage of the skin must be done to avoid friction and reduce the risk of infections. In severe cases, this process can be very painful and long.
• Oral hygiene must be done with extreme care.

All treatment must be directed by a nursing team, they must teach how to care for the patient, all instruments and bandages used must be completely clean. In addition, you must have a great team including doctors, nurses, nutritionists, social workers, among others.

So that the little one can lead a more pleasant life, we recommend:

• Avoid excessive heat.
• Apply lotion to the skin to decrease friction.
• Wear soft clothing.
• Wear gloves at bedtime to avoid scratching.

Making a diagnosis of this disease is not difficult at all, with a simple skin sample analyzed in the laboratory is enough to diagnose it. The problem is that certain countries and hospitals have almost zero knowledge of this condition, which causes children to suffer even more damage. A typical case is the child who is born with butterfly skin and is put in the incubator thinking that it must finish forming. There, with too much heat, his symptoms and pain get worse.

If in general, we talk about the disease we are talking about, it is very difficult to give a specific life expectancy, in the case of butterfly skin it is even more complicated, because depending on the type of epidermolysis, the prognosis will be one or the other.

In the most serious cases, butterfly children usually die in the first years of life , due to malnutrition and anemia that causes them not to be able to eat due to wounds in the esophagus, larynx and mouth. Other less serious cases also have their life expectancy shortened, as many die from skin cancer .

This article is merely informative, at FastlyHeal .com we do not have the power to prescribe medical treatments or make any type of diagnosis. We invite you to see a doctor in the case of presenting any type of condition or discomfort.

If you want to read more articles similar to Butterfly skin: what it is, symptoms and causes , we recommend that you enter our Skin, hair and nails category .