Treatment FAQ

how effective is the treatment for epidermolysis bullosa

by Eliane Quigley Published 2 years ago Updated 1 year ago
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It is also the first trial of gene therapy in children with epidermolysis bullosa. A large 10-year-old wound covering most of the side of a patient's body showed 70% healing, while all other wounds closed completely with treatment.Mar 28, 2022

Medication

Your doctor may recommend a pain medication to help manage pain due to blisters and open wounds on the skin or in other areas of the body. Some types of epidermolysis bullosa cause itching. To help prevent scratching, which can cause fragile skin to tear, your doctor may prescribe medications to help control itch.

Procedures

Perform a skin biopsy, which helps doctors identify which layers of the skin are affected and determine the type of epidermolysis bullosa you have. Order genetic testing to identify specifically which gene mutations you may have. How is epidermolysis bullosa treated?

Self-care

Epidermolysis bullosa (EB) encompasses a group of rare, clinically and genetically heterogeneous genodermatoses with an estimated 500 000 cases worldwide. 1 It is characterized by moderate to excessive fragility of epithelial tissues with prototypic blistering or erosions following minimal trauma (mechanobullous dermatoses).

Nutrition

Currently, treatment for severe forms of EB is focused on promoting wound healing, preventing infection, protecting the skin from trauma, decreasing blister occurrence, minimizing deformities, alleviating pain, attending to nutritional needs, and providing psychological support for the family.

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How do doctors treat epidermolysis bullosa?

How do I know if I have epidermolysis bullosa?

What is epidermolysis bullosa (EB)?

What are the treatment options for EB?

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Is there any cure for epidermolysis bullosa?

There's currently no cure for epidermolysis bullosa (EB), but treatment can help ease and control symptoms. Treatment also aims to: avoid skin damage. improve quality of life.

How is epidermolysis bullosa currently treated?

Medication is often needed to relieve the pain. Antidepressants, medicine used to treat epilepsy, and acetaminophen can be helpful. If the pain is severe, medicine like fentanyl, morphine, or ketamine can be prescribed. Before bathing and wound care, it may be necessary to give pain medication to someone with EB.

How close is a cure for EB?

There is currently no cure for any form of EB, and treatment still focuses largely on wound care by protective bandaging to reduce pain and further damage, and managing complications from the risks of infection, fibrosis and poor nutritional status.

Why is there no cure for epidermolysis bullosa?

There is no cure for any of the subtypes of EB resulting from different mutations, and current therapy only focuses on the management of wounds and pain. Novel effective therapeutic approaches are therefore urgently required. Strategies include gene‐, protein‐ and cell‐based therapies.

How painful is EB?

According to the findings of the MDC researchers, this explains why EB patients are more sensitive to touch and experience it as painful. Even the slightest touch causes a stinging sensation like being stabbed with pins; the body is covered with blisters and the skin is inflamed in many places.

Are you born with EB?

Recessive Dystrophic EB is the most severe, chronic type of EB. Blistering begins at birth or shortly afterwards. Much of the skin is covered in blisters and there is extensive internal blistering.

What are the signs of epidermolysis bullosa?

If your child has EB, you’ll often see signs of this disease, such as blistering or missing skin, when your child is born. The following explains how to get an accurate diagnosis, what treatment may involve, and the prognosis for a child born with EB.

How does EB treatment work?

Treatment for EB consists of caring for wounds, managing pain, preventing new injuries, and watching for and treating complications. At an EB treatment center, you’ll find specialists from different areas of medicine who have experience with EB.

How to diagnose EB in children?

To diagnose EB, a dermatologist will examine the child’s skin closely, looking at the blisters and other signs. Your dermatologist will also examine other parts of the child’s body to see if they are affected.

What causes EB pain?

So many things can cause pain, including the blisters and raw skin, an infection, swallowing, eating, urinating, having a bowel movement, or just moving about. Medication is often needed to relieve the pain.

How often do you change bandages for EB?

Most patients must wear bandages that must be changed every day or every other day. If EB is severe, a patient may need a nurse’s help with wound care. A nurse may go to the home of a patient with EB to help with wound care. Pain management: People with EB are often in pain.

How to treat a wound in a child?

Dehydration: Open wounds can lead to dehydration. This can be treated by giving the child lots of fluids to drink. Sometimes, the child needs an IV to replace lost fluids. Dry skin: Applying a moisturizer every day can help reduce dry skin, which can reduce blistering, pain, and itching.

What to do for EB pain?

Before bathing and wound care, it may be necessary to give pain medication to someone with EB. Adding salt to the bathwater and using dressings that contain pain medicine can also help reduce pain during wound care.

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Diagnosis

Clinical Trials

Lifestyle and Home Remedies

Coping and Support

Medically reviewed by
Dr. Aakash Gupta
Your provider will work with you to develop a care plan that may include one or more of these treatment options.
The treatment goal is to follow a multidisciplinary approach to improve the quality of life. Antibiotics such as tetracycline, surgery and rehabilitation are the main management techniques.
Medication

Analgesics: Over-the-counter painkillers for milder forms and stronger ones for severe conditions.

Ibuprofen . Morphine


Antibiotics: To prevent or treat accompanying infections.

Tetracycline

Procedures

Esophageal dilation: Blisters and scarring can cause narrowing of the esophagus, and widening it helps food passage.

Gastrostomy: Can deliver food directly into the stomach.

Skin grafting: Transplantation of healthy skin.

Orthopedic surgery: To correct fusing hands or abnormally bent joints.

Self-care

Always talk to your provider before starting anything.

  • Do not walk long distances
  • Limit physical activity
  • Wear comfortable shoes and clothing
  • Keep cool as sweating aggravates symptoms
  • Avoid every day bumps, scratches
  • Skin care: promptly treat the wounds to prevent infection
  • Special dental care, nail care, and eye care is needed as they might be affected

Nutrition

Foods to eat:

  • Soft foods such as vegetable soup, fruit smoothie
  • Plenty of fruits such as melon, bananas, kiwi fruit

Foods to avoid:

  • Avoid hard, difficult to digest foods

Specialist to consult

Dermatologist
Specializes in the study of the skin and its disorders.
Dentist
Specializes in the treatment of diseases associated with teeth and gums.
Ophthalmologist
Specializes in the medical and surgical care of the eyes.
Primary care physician
Specializes in the acute and chronic illnesses and provides preventive care and health.
Dieticians
Specialize in the nutritious diet and patient's food habits.

Preparing For Your Appointment

  • A doctor may suspect epidermolysis bullosa from the appearance of the affected skin. He or she will likely have your child undergo laboratory tests to confirm the diagnosis. They may include: 1. Skin biopsy for immunofluorescent mapping.With this technique, a small sample of affected ski…
See more on mayoclinic.org

How Is Epidermolysis Bullosa (EB) Diagnosed?

  • Explore Mayo Clinic studiestesting new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.
See more on mayoclinic.org

How Is Eb Treated?

  • You can take steps at home to care for blisters and prevent new ones from forming. Learn how to care for wounds and provide good nutrition, which are essential to people with epidermolysis bullosa.
See more on mayoclinic.org

Researchers Are Trying to Improve Treatment

  • Treating and preventing blisters and complications of epidermolysis bullosa can be stressful for you, your child and family members. You may find it helpful to share concerns and information with families in similar circumstances. Ask your health care providers about epidermolysis bullosa support groups in your area. If joining a support group isn't for you, ask about counselors, clergy …
See more on mayoclinic.org

What Is The Outlook For Someone Who Has EB?

  • You may be referred to a doctor who specializes in the diagnosis and treatment of skin conditions (dermatologist).
See more on mayoclinic.org

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