Butterfly disease: A condition that makes the skin as sensitive as butterfly wings

Disease name: Epidermolysis bullosa (EB) or “butterfly disease”

Affected Populations: It is estimated that the butterfly disease is affected about 1 in 50,000 childrenif you add up all the subtypes of the disease. EB is equally common in men and women and across all races and ethnicities.

Causes: Butterfly disease is a group of rare hereditary diseases This causes the skin to become very sensitive and blister easily. There are around 30 subtypes of butterfly diseaseand these are divided into four main groups, which differ depending on the area of skin affected.

The most common form of the disease, which affects 70% of EB patients, is called epidermolysis bullosa simplex (EBS). Patients with EBS carry genetic mutations that affect the outermost organs layer of their skincalled epidermis. EBS is usually an autosomal dominant inherited disorder, meaning children who inherit just one copy of the mutated gene from a parent will develop it. The disease is rare is autosomal recessivein which a child requires two copies of the gene – one from each parent – to develop.

Related: “Butterfly disease” makes the skin incredibly fragile, but a new gene therapy is helping to heal it

Symptoms: Patients usually begin to develop symptoms of butterfly disease at birth or in early childhood. Symptoms can vary from mild to severe and differ depending on the subtype of the disease. Common, universal symptoms of EB include: Skin that blisters easilyespecially on the hands and soles of the feet. These areas of the body also often have thickened, scarred skin. Mild forms of butterfly disease may improve with age And are not fatalbut patients with severe forms usually do not live past 30 years. Because severe cases can lead to life-threatening infections and damage to internal organs.

A diagram showing the layers of skin

Butterfly disease affects different parts of the skin depending on the subtype of the disease. (Image credit: Flash Vector via Getty Images)

Treatments: Currently exists No cure for butterfly disease. However, appropriate wound care – such as removing blisters and using non-adhesive bandages and bandages to cover the wounds – can help patients manage their symptoms. Medication can also be taken relieves itching and pain The blisters may be associated with antibiotics, and any associated bacterial infections can be treated with antibiotics. Some patients surgery may also be required if the disease causes narrowing of the esophagus or if there are problems using the hands due to excessive scarring.

Approval by the US Food and Drug Administration (FDA) took place in 2023. a new gene therapy gel to treat a certain type of butterfly disease in patients who are at least 6 months old. This form of the disease, called dystrophic epidermolysis bullosa, is caused by mutations in a gene that codes for a type of collagen in the skin. The gene therapy, known as Vyjuvek, delivers working copies of the collagen gene into the patient’s cells.

The same gene therapy was also adapted for eye drops in 2023. She was deployed to help Restore a teenager’s vision who was officially blind due to scarring of his eyes caused by butterfly disease.

The FDA has also granted approval another gel called Filsuvezfor the treatment of wounds in patients aged 6 months and over. The gel made from birch bark is only approved for certain types of lepidopteran diseases.

This article is for informational purposes only and does not constitute medical advice.

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