Epidermolysis Bullosa

Medically Reviewed by Dr. K on 10 March 2021

What Is Epidermolysis Bullosa?

Epidermolysis bullosa is a rare hereditary disorder that causes skin to be so delicate that it tears or blisters even when touched lightly. Since their skin appears as delicate as a butterfly wing, children born with it are often referred to as “Butterfly Children."

Mild forms can improve over time. Extreme cases, on the other hand, can be debilitating, cause other significant health problems, and even be life-threatening.

If you have this disorder, you will need extra care in order to maintain the protection of your fragile skin.

Types

Epidermolysis bullosa is divided into five forms. The variety you have is determined by the location of your blisters.

Epidermolysis Bullosa Simplex: It is the most common form which first appears in newborns. The palms of the hands and the soles of the feet are the most often affected areas.

Junctional epidermolysis bullosa: This is a more serious type that causes blistering in the deep layers of the skin, and it even starts in infants.

Dystrophic epidermolysis bullosa: If you have this kind, your skin lacks collagen to keep it intact, or the collagen you do have is ineffective. This means the skin’s layers aren’t properly sealed together. This form does not often appear until early childhood.

Kindler syndrome: Since blisters occur on various layers of the skin, this is a mixed disorder. When the skin is exposed to the light, it can also create patchy colour changes.

Epidermolysis bullosa acquisita: Blisters appear on the hands and feet, as well as mucous membranes such as the lips, in this form.

 

Causes

Epidermolysis bullosa runs in families in almost all cases. You would get it if you inherited those gene defects from your ancestors.

There is one exception to this rule. The only variety that is not hereditary is epidermolysis bullosa aquisita. It occurs as a result of an immune system defect.

 

Symptoms

Epidermolysis bullosa symptoms typically occur in infants or toddlers. The primary symptom is painful skin blisters. They can form on any part of the body. They can also form on the eyes, the throat, the stomach, and the bladder. More complications arise if the blisters get swollen or scar the skin.

Diagnosis

Your doctor can take a small sample of skin and send it to a lab where experts can examine it under a microscope to confirm the diagnosis.

 

Treatment

Epidermolysis bullosa has no known cure. However, there are therapies available.

If you have a bad case, you’ll treat the skin the same as you would if you had a burn. You’ll need to learn how to heal wounds on a regular basis, as well as how to bandage and secure the infected areas.

Medication to assist with pain relief can also be prescribed by the doctor.

In certain circumstances, surgery can be needed. Your doctor will separate your fingers and toes if you have blisters that have glued them together. Alternatively, if the oesophagus, the tube connecting your mouth and stomach, gets too scarred to enable you to feed, you may have surgery to enlarge it.

Eating will also be uncomfortable for some people with epidermolysis bullosa. Under any case, your doctor may recommend a feeding tube so that food can be delivered directly to your stomach.

 

Tips for At-Home Care

You must take extra care of your skin in order to avoid blisters.

Reduce the amount of pressure. To keep the skin soft and reduce friction, apply lotion. Using just non-sticky dressings to protect cuts, or loosely seal with wrapped gauze again. Wear clothes and materials that are loose-fitting with no patches, short sleeves, or seams.

Blisters should be drained. These will fill with fluid and become sick if not treated. Your doctor will be able to teach you how to drain them properly.

Keep your composure. Bathe in water that is no hotter than room temperature. Stop heat and humidity as much as possible by staying in the air conditioning.

Understand the symptoms of illness. If the skin becomes sick, it can turn red or feel hot to the touch. You can also find pus or a yellow discharge crusting at the site, a red streak under the skin, or fever or chills. Contact your doctor right away if you have any of these symptoms. Antibiotics may be needed.

Examine your diet. Iron, selenium, and vitamin D deficiency are common in epidermolysis bullosa patients. Your doctor may advise you to consult a nutritionist about increasing your intake of these vitamins and minerals.

Find support. Speak with the doctor or a trusted family or friend. You may also find it beneficial to discuss your thoughts with a therapist or at a local community support group.

Sources

Referenced on 10.4.2021

  1. Mayo Clinic, “Epidermolysis Bullosa.”
  2. Dystrophic Epidermolysis Bullosa Research Association of America, “What Is EB?”
  3. National Institute of Arthritis and Musculoskeletal And Skin Diseases (NIAMS), “What is Epidermolysis Bullosa?”
  4. National Organization for Rare Disorders, “Epidermolysis Bullosa.”
  5. https://www.webmd.com/skin-problems-and-treatments/epidermolysis-bullosa-what-is

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