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What is Butterfly Skin? Symptoms, Diagnosis and Treatment

Butterfly skin, also known as epidermolysis bullosa, is the name given to a group of diseases characterized by skin fragility. It’s a very rare skin disorder that affects only a few thousand people worldwide. In this article, we’ll show you what it is, its types, and what options exist to treat it.

According to researchers, only 19 cases per million inhabitants are reported in the United States. The symptoms are very varied, as well as their evolution. It’s one of the most enigmatic conditions, although this does not imply a total lack of knowledge on behalf of scientists. In the following article, we’ll tell you everything you should know about butterfly skin.

The characteristics of butterfly skin

Although the characteristic lesions of epidermolysis bullosa are blisters, these patients have a tendency to develop several skin problems.

As experts indicate, butterfly skin is characterized by mucocutaneous fragility accompanied by blisters that develop due to minimal trauma. In fact, lesions can appear due to seemingly harmless actions such as exposure to the sun for a few minutes, rubbing the skin with an object, scratching, or using an adhesive bandage.

Depending on the level of tissue separation, scientists distinguish three types of butterfly skin: simple, junctional, and dystrophic. Let’s briefly take a look look at the characteristics of each of these:

  • Epidermolysis bullosa simplex: this is the most frequent variant of them all. It usually only affects the palms and soles and always affects the outer layer of the skin (epidermis). The blisters are typically mild and, when they heal, leave no visible scars.
  • Junctional epidermolysis bullosa: People who develop this type of butterfly skin usually do so from childhood with severe lesions. It affects the collagen and laminin of the skin on the whole body (the joints, hands, feet, etc.). However, it’s not uncommon for children with this variant to develop blisters on the vocal cords.
  • Dystrophic epidermolysis bullosa: This occurs due to the absence of a gene that provides a type of collagen required for the skin to gain strength. It has two main subtypes: Pasini’s disease and pruritic epidermolysis bullosa.

Overall, most cases of butterfly skin are hereditary, although evidence indicates that it can also be acquired. Apart from this, having a family history of the disease potentially raises a person’s chances of developing it in childhood or at some point later in life.

Symptoms of epidermolysis bullosa

Butterfly skin is a very heterogeneous condition. This is not only because the clinical picture of the three types is different, but also because patients with the same variant don’t develop it with the same intensity or signs.

Generally, we can highlight the following symptoms of epidermolysis bullosa:

  • Fragile skin that breaks and blisters easily (with slight friction with an object or even with a temperature change, for instance).
  • Blisters inside the oral cavity or throat.
  • Malformation of the fingernails and toenails (they’re usually thicker).
  • Thick skin, especially on the hands and feet.
  • Itching and burning on the skin surface.
  • Atrophic scarring after the blisters have disappeared.
  • Blisters on the scalp.
  • Swallowing problems (dysphagia) that can evolve into malnutrition due to the refusal to eat food.
  • Recurrent infections due to exposure to blisters.

These are the general symptoms of butterfly skin. However, of course, many more can develop. For example, constipation, joint pain, malformation of the fingers and toes (they can come together in scarring), sepsis, and so on can all also be a part of this rare condition.

Diagnosis of butterfly skin

The best method of making an accurate diagnosis is by skin biopsy. Occasionally, an immunohistochemical study may be necessary.

Butterfly skin is a very rare disease, and mild cases are often easily confused with other types of skin diseases. In any case, the standard procedure for its diagnosis is to perform a skin biopsy for immunofluorescent mapping.

This is a procedure in which a skin sample is removed from the affected area for a special examination so that the different layers of the skin can be studied. This type of biopsy also allows identifying the proteins involved in the layers analyzed.

If the condition is suspected to be hereditary, the specialist will also perform genetic testing.

Treatment for epidermolysis bullosa

There is currently no cure for butterfly skin. However, a couple of options are available to make the condition much more tolerable for those who have it. In principle, it all depends on the severity with which it has developed and the long-term prognosis.

For example, small changes in a person’s day-to-day habits such as wearing looser clothing, modifying their diet, or being careful with household chores may be enough in mild cases. Medication is only recommended for moderate to severe cases, especially when it is needed to treat wounds that may develop into infections and pain.

Scientists are investigating alternative treatment options for the future, such as protein replacement therapy and bone marrow transplantation. Some patients require surgery to widen their esophagus, restore mobility, get skin grafts, and even intervention with feeding tubes. They must also learn to heal and dress wounds properly.

Although the options are limited at the moment, scientists hope that there will be treatment alternatives that will reduce the symptoms and the progression of the disease in a couple of decades. Meanwhile, for the time being, it’s recommended to seek medical assistance if you notice any symptoms so that you can get appropriate medical treatment depending on the intensity and type of variant you have.

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