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Butterfly skin

The butterfly skin is a rare disease characterized by extreme fragility of the skin. Thus, those who suffer from it, suffer daily the formation of wounds and blisters on the body at the slightest friction, and even the involvement of mucous membranes. To this day, this disorder has no cure.

The latest studies suggest that 1 in 227 people is a carrier of some of the genetic mutations that cause the disease. The current incidence is 18 affected births per million births. In Spain it is estimated that approximately 500 people suffer from butterfly skin.

What is butterfly skin?

Butterfly skin is defined as a rare genetic disease that causes extreme fragility of the skin and mucosa. At the slightest friction with any part of the face or body, lesions appear in the form of wounds and blisters, which take several days to heal.

In those who suffer from this disease, the proteins that allow the skin to have its own resistance, either do not exist, or they do not work properly. Thus, depending on the type of protein affected, butterfly skin affects the person in a certain way.

This disorder does not carry any intellectual limitation . Even so, in some cases the internal mucous membranes can be affected, which causes serious difficulties both in eating and swallowing.

The effects of this disease go much further. Those who suffer it in many cases suffer serious social and psychological consequences . Butterfly skin negatively affects the way people relate to their environment, and often causes social rejection.

In the case of children, this disorder causes a high rate of school absenteeism since medical visits are frequent, as well as hospital admissions due to, for example, a wound that has not healed properly and has caused an infection . The same occurs with adults, among whom the absenteeism rate is very high.

Types of butterfly skin

Three subtypes of the disease are distinguished.

  • Simple: in this case, the break occurs in the most superficial layer of the skin, which is called the epidermis. Blisters and wounds that occur at the slightest friction heal relatively easily, without tissue loss.
  • Junctional: a type of butterfly skin in which blisters and wounds appear between the inner and outer layers of the skin. It is the least frequent type of all.
  • Dystrophic: wounds and blisters originate in the deepest layer of the skin, called the dermis. When the various injuries heal, retractions in the muscles and joints occur, so that movement is seriously hampered over time.

It is not possible for one type of butterfly skin to evolve into another. That is, if a baby is born with a mild form, such as simplex, they will never develop a more severe form, such as dystrophic.

Symptoms of butterfly skin

The butterfly skin is a disease whose symptoms are very noticeable and clear, so it is relatively easy to diagnose. Those who suffer from it have extremely fragile skin, especially on the hands and feet.

One of the signs of this disorder is the appearance of blisters and the formation of scars, even on the scalp. There are also dental problems, such as cavities, because the teeth do not finish forming properly. Similarly, nails are also not formed.

Patients suffer from blisters in the mouth and throat, which makes swallowing difficult and in many cases leads to malnutrition.

Diagnosis and treatment

Medical personnel may suspect a newborn baby of butterfly skin from a number of physical symptoms . In this case, he will take a small sample of the child’s skin and examine it later in the laboratory. In this way, it is diagnosed if you suffer from this disorder and, if so, what form it is.

So far this is a disease that has no cure . Therefore, the various treatments that exist are purely palliative. The sick require daily treatment, which generally consists of cures for the lesions. In addition, there are specific ointments and creams to relieve the sensation of itching and burning.

You may also be interested in:   Atopic dermatitis in children

Those who suffer from dystrophic butterfly skin require more meticulous care, very similar to that received by those who suffer second or third degree burns. In these cases, they must go to their health center on a daily basis so that a nurse can perform the cure.

Today there are several avenues of research to find a cure for the disease. One of the most recent studies dates from 2017 in Germany by the Italian dermatologist Michele De Luca and his team. They managed to make a new skin for a 7-year-old boy from his own cells .

Healing process

  • Environment: the first step before starting the healing process is to prepare the right environment for it. It is important to take care of the temperature and the light. Also, the space must be completely clean.
  • Material: next, you must prepare the cure material and wash your hands meticulously, with plenty of warm water and pH neutral soap.
  • Removal: the first thing is to proceed with the removal of the patient’s clothing, bandages and dressings.
  • Bath: through a warm water bath, the dressings that have stuck to the skin are removed.
  • Wounds: then the wounds are cleaned and decontaminated.
  • Hydration: and finally, healthy skin is hydrated with specific products, dressings are applied and bandaged again.

Complications

  • Syndactyly: Syndactyly is the fusion of two or more fingers between them. Although it is a rare condition in people, it can occur in those who suffer from this disease.
  • Dysphagia: Dysphagia is the term used to refer to difficulty swallowing. This is due to the formation of blisters in the throat.
  • Malnutrition: When people with butterfly skin have blisters in their mouth, eating is difficult, so malnutrition is a relatively common complication. In the case of children, nutritional problems can cause a delay in their growth both physically and mentally.
  • Constipation : Due to the appearance of blisters in the anal area, evacuating the stool can be difficult. Constipation can also be due to insufficient intake of fluids, as well as foods rich in fiber.
  • Kidney failure: Acute kidney failure occurs when one or both kidneys lose the ability to filter waste from the bloodstream.
  • Muscular dystrophy: Muscular dystrophy is a group of progressive diseases that cause the weakness of various muscle groups in the human body.
  • Cardiomyopathy: Another possible complication of butterfly skin is cardiomyopathy. A disorder in which the heart greatly reduces the ability to pump blood to the rest of the body.

Is butterfly skin contagious?

Contrary to what many believe, this is not a contagious disease . It is a genetic disorder, so those who suffer from it are born with it. There is no way of contagion, not even having direct contact with a person with the disease.

How to care for babies and children with butterfly skin?

For those parents whose children suffer from this disease, there are a number of steps they must take.

  • Caution: it is very important to treat babies and children with great care. In no case should they be lifted under the arms. To do this, it is best to place them on a soft material and hold them carefully behind the neck and under the buttocks.
  • Diaper area: When babies with butterfly skin use a diaper, it is advisable to avoid wet wipes for cleaning. In addition, it is recommended to remove the elastic bands so that they do not cause injury to your skin.
  • Humidity: maintaining optimal humidity levels in the home is essential. A dry environment favors the dryness of the skin and, therefore, the formation of wounds and blisters.
  • Activity: the fact of suffering from butterfly skin does not imply that children should stay at home. The role of parents is to encourage them to be active by practicing activities such as swimming.

The butterfly skin is a disease of a serious nature, characterized by the formation of wounds and blisters. A disorder that today has no cure, but requires daily care.

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Hello Readers, I am Nikki Bella a Psychology student. I have always been concerned about human behavior and the mental processes that lead us to act and think the way we do. My collaboration as an editor in the psychology area of ​​Well Being Pole has allowed me to investigate further and expand my knowledge in the field of mental health; I have also acquired great knowledge about physical health and well-being, two fundamental bases that are directly related and are part of all mental health.

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