Ever wondered why you have delicate skin? This can happen because you have butterfly skin disease or epidermolisis bullosa, which is very rare.
Epidermolisis Bulosa, also known as butterfly skin disease in colloquial language, a rare genetic condition that makes your skin very delicate. While symptoms usually appear in infants or young children, sometimes, signs begin to appear later in life. The inherited condition can affect adults as well as adults. Currently, there is no cure for the disease, but there are treatment options that help relieve symptoms. Efforts can also be made to prevent complications such as infection.
What is butterfly skin disease?
Butterfly skin disease or epidermolisis Bulosa (EB) is a heritated disease of connective tissues that affect the skin and make it extremely delicate. “Just a minor friction or skin trauma can cause blisters and skin tears,” says dermatologist Dr. Khushu Jha,
It makes the skin very delicate and delicate that is found in the butterfly feathers. In addition to the skin, the disease affects the mucous membrane of the body. However, it can be severe, and can affect the food pipe and urinary bladder. About 50 living births in 1 million are diagnosed with butterfly skin disease in the world, according to research published in the world Medical Journal, Armed Forces India,
What are the symptoms of butterfly skin disease?
Symptoms of butterfly skin disease are quite variable, which is based on the type and its severity. But some more common symptoms include:
- Skin blisters Just the skin with a little friction and trauma
- Painful wounds on the skin -the mucous membrane of the body such as mouth, eyes or throat
- Along with the skin on the hands -the legs become thicker, which is also known as hypercratosis
- Broken nails of the fingers of the hands or toes
- Tooth decay or other dental problems
- Difficulty swallowing food due to esophagal strictness
- Fingers
- Easy infection in open wounds
- Low hemoglobin levels due to anemia
- Climax
What is the cause of butterfly skin disease?
“The main reason behind this condition is in genetic mutations in genes that serve to produce proteins that serve to keep the layers of the skin together.” This includes a role playing a role, including keratin, collagen VII, laminine and integins. The inherited genetic mutation leads to either the absence or mistake of such proteins, and the skin develops easy blisters, ”experts explain.
What are the types of butterfly skin disease?
There are four major types of EB:
- Epidermolisis bullosa simplex (EBS): It is the lightest form of EB that affects the outer layer of the skin, and in terms of friction, it leads to blisters on hands and feet. This includes about 70 percent of all butterfly skin disease cases, as published according to research. Statpearls,
- Junctional epidermolisis bullosa (JEB): It is a moderate to severe type of EB, which affects both the mucous membrane along the skin. In infants, it often takes a serious turn.
- Distrophic epidermolisis bullosa (DEB): This type of EB affects deep skin layers, causing skin signs and deformity. Dr. “It can also progress for cancer in later life,” says Jha.
- Kinder syndrome: It is a rare and mixed type of EB that tipped the skin, along with the skin -the skin’s sensation for the atrophy of the skin, which is the skin’s thinner, which is the skin’s thinner.
What is the life expectancy of people with butterfly skin disease?
The life expectancy of people with this situation depends on the type and severity:
- There is a normal life expectancy when the EB -suffering people are dealt with with the care of Epidermolisis Bulosa Simplex (EBS).
- Severe pockets and severe deb are highly uncomfortable. “Pocket Herlitz is very dangerous for sub -infants, with a survival rate of up to one year.
- Serious DEB also increases the risk of squamous cell carcinoma (a type of cancer) with small life expectancy up to 30 and 40s.
How to treat butterfly skin disease?
There is no definite treatment for butterfly skin disease, but treatment options are:
- A gel with birch bark extracts can be used to treat diastrophic epidermolisis bullosa and junctional epidermolisis bullosa according to the UK. National heath service,
- Since the skin is extremely delicate in people with this condition, wound care is important. Non-stick strips can be helpful in avoiding skin trauma during changes in dressing. Antiseptics or sterile saline help prevent clean wounds and infections. Silicon-based dressing or special wound gels can be used Rapid treatmentTo reduce any discomfort, topical painkillers are sometimes applied before the dressing change.
- Open wounds suffer from bacterial infections, which can be severe. Experts say, “topical antibiotics such as meupirocin or fusidic acid can be applied to infected areas. In more severe cases, oral or IV antibiotics can be determined by your doctor,” experts say.
- Due to painful blisters in the mouth or esophagus, food can be difficult. “Soft, high-calorie diets are good options to support wound healing,” experts say.
- In cases where oral feeding is very painful or unsafe, feeding tubes (gastrostomy or nasogastric) can be inserted.
- Supplements, especially iron, zinc and vitamins A, C, and E, can help increase skin uplift and immunity.
Butterfly skin disease is genetic, so it cannot be prevented after birth. There is also no cure, but there are treatments that can help manage symptoms and prevent infection.
Related question
Is the butterfly a gene therapy for skin disease?
Yes, gene therapy is subject to development and showing promises. In particular, a topical gene therapy called Vyjuvek (Beremagin Geperapavec) was approved for dystrophic epidermolisis Bulosa (DEB) due to mutation in the col7a1 gene. This causes the working copies of the gene directly to the wounds.
Are adult butterfly affected by skin disease?
Yes, although the EB usually manifests in infancy or childhood, some miller forms may be uncontrolled until adulthood or remaining in adult life. Lightly the hands and feet can be a lifelong blister.
