Butterfly skin is the generic name for a group of skin conditions. We show you their types, characteristics and treatment.
Butterfly skin, also known as epidermolysis bullosa or epidermolysis bullosa, is the name given to a group of diseases that are characterized by the fragility of the skin. It is a very rare skin disorder that only affects a few thousand people worldwide.. We show you what it is, what its types are and what options exist to treat it.
In accordance with the researchers, in the United States only 19 cases per million inhabitants are reported. The symptoms are very varied, also its evolution. It is one of the most enigmatic conditions, although this does not imply a total ignorance on the part of scientists. In the following lines we show you everything you should know about butterfly skin.
Butterfly skin characteristics
As indicated The experts, butterfly skin is characterized by mucocutaneous fragility that is accompanied by blisters that develop due to minimal trauma. Indeed, lesions can appear from harmless actions such as exposure to the sun for a few minutes, rubbing the skin with an object, scratching or using adhesive tape.
According to the level of separation of the tissues, the scientists distinguish three types of butterfly skin: simple, junctional, and dystrophic. Let’s briefly see the characteristics of these:
- Simple epidermolysis bullosa: it is the most frequent variant of all. It usually only affects the palms and soles, and it always does so on the outer layer (epidermis). The blisters are usually mild and when they heal they do not leave visible scars.
- Junctional epidermolysis bullosa: patients who develop this type of butterfly skin do so from childhood with serious injuries. It affects collagen and laminin, so it can affect the entire body (joints, hands, feet, and so on). It is not uncommon for children to develop vocal cord blisters with this variant.
- Dystrophic epidermolysis bullosa: It manifests itself due to the absence of a gene that contributes a type of collagen that the skin requires to acquire strength. It has two main subtypes: Pasini’s disease and pruritic epidermolysis bullosa.
Most cases of butterfly skin are inherited, although the evidence indicates that can also be purchased. Apart from this, having a family history of the disease potentially increases the chances of developing it in childhood or at some point in life.
Symptoms of epidermolysis bullosa
Butterfly skin is a very heterogeneous condition. Not only because the clinical picture of the three types is different, but because patients with the same variant do not develop it with the same intensity or signs. In general, we highlight the following symptoms of epidermolysis bullosa:
- Fragile skin that breaks and blisters easily (with slight friction with an object, even with a change in temperature).
- Blisters inside the oral cavity or throat.
- Malformation of the nails of the feet and hands (they are usually thicker).
- Thick skin, especially on the hands and feet.
- Itching and burning on the surface of the skin.
- Atrophic scarring after the blisters have disappeared.
- Blisters on the scalp.
- Swallowing problems (dysphagia). This can evolve into malnutrition due to rejection of food.
- Recurring infections due to exposure of the blisters.
These are the general symptoms of butterfly skin, although of course many more can develop. For example, constipation, joint pain, malformation of the fingers and toes (they can join in the scarring), septicemia and so on.
Butterfly skin diagnosis
Butterfly skin is a very rare disease, and mild cases are often easily confused with other types of skin pathologies. The standard procedure for its diagnosis is to perform a skin biopsy for immunofluorescent mapping.
It is a procedure in which a sample of skin is removed from the affected area to examine it in a special way, so that the different layers of the skin can be appreciated. This type of biopsy also makes it possible to identify the proteins involved in these analyzed layers.
If the condition is suspected to be hereditary, the specialist will also perform genetic tests.
Treatment for epidermolysis bullosa
There is currently no cure for butterfly skin. However, there are a couple of options available to make the condition much more tolerable for patients. In principle, it all depends on the severity with which it has developed and the long-term forecasts.
For example, small changes in day-to-day habits such as wearing looser clothes, modifying your diet or being careful with household chores may be sufficient in mild cases. For moderate or severe cases, the intake of medications is preferred, especially to treat wounds that can evolve into infections and pain.
The scientists they are investigating alternative treatment options for the future, such as protein replacement therapy and bone marrow transplantation. Some patients require surgery to widen the esophagus, restore mobility, skin grafts, and even intervention with feeding tubes. They must also learn to heal and bandage wounds.
Although at the moment the options are limited, it is expected that in a couple of decades there will be treatment alternatives that will reduce the symptoms and the progression of the disease. At the moment it is recommended to seek medical assistance before the development of symptoms, so you can choose to start a treatment depending on the intensity and type of variant manifested.
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