Ever Trend Zone Have you ever wondered why you have fragile skin? It may be because you have very rare butterfly skin diseases or epidermal diseases.
Epidermal bulls, also known as epidermal skin skin disease, are also rare genetic conditions that make the skin extremely fragile. Symptoms usually appear in babies and young children, but sometimes signs begin to appear later in life. Inherited states can affect not only adults but also teenagers. Currently, there is no cure for this disease, but there are treatments that can help relieve symptoms. You can also make efforts to prevent complications such as infections.
What is Butterfly Skin Disease?
Butterfly skin disease or epidermis (EB) is a hereditary disease of connective tissue that affects the skin and makes it extremely fragile. “Mild friction or trauma to the skin can lead to blisters and tears in the skin,” says Dr. Kushbu Ja, dermatologist.
It makes skin resemble butterfly wings very delicate and fragile. Apart from the skin, this disease affects the body’s mucous membranes. However, it can become severe and affect the food pipe and bladder. A study published in the Indian Army’s medical journal shows that about 50 out of 1 million births worldwide have been diagnosed with butterfly skin disease.
What are the symptoms of butterfly skin disease?
The symptoms of butterfly skin disease vary greatly depending on the type and its severity. However, some of the more common symptoms are:
- Skin blisters are caused by slight friction and trauma.
- Painful skin pain and mucous membranes of the body, including the mouth, eyes, and throat
- Not only does it become thicker legs, but it is also known as emollacia.
- Broken nails on fingers or toes of the feet
- Cataract or other dental problems
- Difficult to swallow food due to esophageal stenosis
- Scars on fingers and toes
- Simple infections in open wounds
- Low levels of hemoglobin due to anemia
- Extremely lethargic
What causes skin diseases in butterflies?
“The main cause behind this condition is the genetic variation in the genes that work to produce proteins that function to hold the skin layer together. Hereditary genetic variations lead to either the lack of such proteins or the faults within them, facilitating the development of blisters in the skin,” the expert explains.
What are the types of butterfly skin diseases?
There are four main types of EB.
- epidermolyis lullosa simplex (EBS): It is the lightest form of EB that affects the outermost layer of the skin, which in the case of friction leads to blisters in the hands and feet. According to a study published in Statpearls, it consists of about 70% of cases of butterfly skin disease.
- Bull of the epidermal cells at the junction (JEB): It is a medium to severe type of EB and affects both the skin and mucous membranes. In babies, serious turns are often required.
- Dystraphytic epidermal bull (deb): This type of EB affects the deeper skin layer, leading to scarring and deformation of the skin. “This could even progress to cancer in later life,” says Dr. Jha.
- Kindler syndrome: It is a rare and mixed EB that contains skin swelling, skin sensitization, skin atrophy, skin thinning, skin sensitization.
What is the average life expectancy for a person with butterfly skin disease?
The average life expectancy of people in this condition depends on their type and severity.
- People who suffer from mild types of EB, such as epidermal bull simplex (EBS), have normal life expectancy when paid attention.
- Severe forms, namely JEB and severe fat, are extremely uncomfortable. “The Jeb Herlitz subtype is extremely dangerous for babies, with survival rates of just one year.
- Severe DEB also increases the risk of squamous cell carcinoma (a type of cancer) with shorter life expectancy in people in their 30s and 40s.
How to treat butterfly skin diseases?
There is no definitive treatment for butterfly skin disease, but there are treatment options.
- Using gels with birch bark extracts, according to the UK National Heath Service, deformed epidermal bulls and junctional epidermal bulls in people over six months can be treated.
- Wound care is very important because the skin of a person in this condition is very delicate. Non-stick bandages help to avoid skin trauma during dressing changes. Preservatives or sterile saline help to clean the wound and prevent infection. Silicone-based dressings or special wound gels can be used for faster healing. To minimize discomfort, topical painkillers may be applied before changing the dressing.
- Open wounds can be severe as they are suffering from a bacterial infection. “Top antibiotics such as mupirocin and fusidic acid can be applied to infected areas. In more serious cases, oral or IV antibiotics may be prescribed by a doctor,” experts say.
- It can be difficult to eat due to painful blisters in the mouth or within the esophagus. “A soft, high-calorie diet is a good option to support wound healing,” says the expert.
- If oral feeding is too painful or is not safe, a feeding tube (gastric somy or nasogastric) may be inserted.
- Supplements, especially iron, zinc, vitamins A, C, and E, help regenerate the skin and improve immunity.
Butterfly skin diseases are genetic and cannot be prevented after birth. There is no treatment, but there are treatments that can help you manage your symptoms and prevent infection.
Related FAQs
Is there a gene therapy for butterfly skin disease?
Yes, gene therapy is under development and shows promise. In particular, a local gene therapy called vyjuvek (Beremagene geperpavec) was approved for dystrophic epidermal bulls (DEBs) caused by mutations in the Col7a1 gene. This delivers a working copy of the gene directly to the wound.
Are adults affected by butterfly skin disease?
Yes, EB usually appears in infancy or early childhood, but some mild forms may not be diagnosed until adulthood or may stick to adulthood. Mild people can cause lifelong blisters in the hands and feet.
