Understanding EB | No Babies Blister

The Basics

What is Epidermolysis Bullosa?

Epidermolysis Bullosa (EB) is a group of rare, genetic blistering skin diseases that cause the skin — and often the mucous membranes lining the body's internal organs — to blister and break down at the slightest friction or pressure.

In healthy skin, proteins called keratins and laminins act like molecular "glue," binding the outer skin layer (epidermis) to the layer beneath it (dermis). In EB, a genetic mutation disrupts this bonding system, meaning the skin layers separate at the lightest touch.

Even a loving hug, wearing clothes, eating food, or normal daily activities can cause excruciating blisters, open wounds, scarring, and internal damage. It is called the "butterfly disease" because children's skin is as fragile as a butterfly's wings.

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Survival in Severe EB

Many babies with the most severe form do not survive their first birthday

80%

Skin Loss Possible

In severe cases, blisters can cover up to 80% of the body's surface

Major Types

EB Simplex, Junctional EB, Dystrophic EB, and Kindler Syndrome

<30

Years Life Expectancy

Severe forms often limit lifespan to under 30 years with extreme suffering

Recognizing EB

Symptoms & Signs

EB affects every system of the body, not just the skin. Here's what EB children experience daily.

Severe Blistering

Large, fluid-filled blisters form at the slightest friction — from clothing seams, medical equipment, or even gentle touch. These blisters burst, creating open, raw wounds.

Extreme Pain

Every wound, every bandage change, every movement causes excruciating pain. Standard pain medications often fail to fully control EB pain, requiring specialized management.

Chronic Infections

Open wounds become constant infection sites. Sepsis (bloodstream infection) is a leading cause of death in EB patients. Ongoing antibiotic treatment is essential.

Fusion of Fingers & Toes

Repeated blistering between digits causes scar tissue that fuses fingers and toes together — a painful condition called pseudosyndactyly requiring surgery.

Feeding Difficulties

EB affects the lining of the mouth, throat, and digestive tract. Eating is painful; swallowing is difficult. Children require specialized nutrition and feeding therapies.

Skin Cancer Risk

Chronic wounding and scarring dramatically increases the risk of aggressive squamous cell carcinoma — one of the leading causes of death in older EB patients.

Classification

The Four Types of Epidermolysis Bullosa

EB is classified by where the skin splits and which gene is affected. All types cause suffering; severity varies significantly.

EB Simplex

Mild–Moderate

The most common form, where blistering occurs within the top layer of skin (epidermis). Usually affects the hands and feet most severely. Generally non-scarring but can be disabling.

Caused by mutations in keratin genes (KRT5 or KRT14)
Blisters triggered by heat and friction
Skin generally heals without scarring
Affects daily activity and mobility

Junctional EB

Severe

Blistering occurs at the junction between the epidermis and dermis. Often severe and life-threatening. The most severe subtype (Herlitz) is frequently fatal in early childhood.

Mutations in laminin or collagen XVII genes
Affects internal organs and airways
Many infants do not survive the first year
Requires intensive daily medical management

Dystrophic EB (DEB)

Critical

Blistering in the upper dermis due to abnormal or absent collagen VII. Causes severe scarring, digit fusion, and dramatically elevated skin cancer risk. Both recessive and dominant forms exist.

Mutation in the COL7A1 gene
Progressive scarring and contractures
High risk of aggressive squamous cell carcinoma
Can involve esophagus, requiring dilation procedures

Kindler Syndrome

Moderate–Severe

A rare form involving blistering at multiple levels of the skin simultaneously. Caused by mutations in the FERMT1 gene. Associated with photosensitivity and increased cancer risk over time.

FERMT1 gene mutation (kindlin-1 protein deficiency)
Sun sensitivity and easy bruising
May improve with age in some cases
Risk of gastrointestinal complications

Daily Reality

What EB Children Face Every Day

For most children, getting dressed, going to school, and playing are simple joys. For EB children, these moments are filled with pain and fear.

4+ Hours of Daily Bandaging

Bandage changes can take 4–6 hours each day. The process of removing and reapplying bandages is agonizing, as bandages adhere to wounds and rip the skin upon removal.

Education Barriers

Many EB children cannot attend regular school due to infection risk, pain levels, and physical limitations. Bullying and social isolation are significant challenges.

Catastrophic Medical Bills

Bandages alone can cost $1,000–$3,000 per month. Medications, surgeries, specialist visits, and nutritional supplements create devastating financial burdens for families.

Severe Nutritional Challenges

Pain while eating limits caloric intake. Wounds require enormous energy to heal. EB children often need therapeutic nutrition formulas to maintain healthy weight.

Emotional & Mental Health

Chronic pain, disfigurement, and social isolation lead to severe depression and anxiety. Both children and caregivers experience profound psychological trauma.

Caregiver Exhaustion

Parents and caregivers dedicate nearly all waking hours to wound care. Many cannot maintain employment, leading to financial collapse and family stress.

Medical Care

Current Treatments & Medical Care

While there is currently no full-body cure for EB, several treatments exist to manage symptoms, reduce pain, and prevent complications.

Wound Care & Bandaging

Non-stick, silicone-coated bandages changed daily or multiple times daily. Specialized foam padding and compression wraps protect fragile skin.

Pain Management

Opioid and non-opioid medications, topical anesthetics during bandage changes, and oral pain protocols. Pain is often severe enough to require specialized pediatric pain teams.

Infection Prevention

Prophylactic antibiotics, antiseptic wound cleansers, and regular infection monitoring. Sepsis prevention is a primary clinical priority.

Nutritional Support

Therapeutic nutrition formulas, feeding therapy, and gastrostomy tubes for children unable to eat adequately due to mouth and throat involvement.

The Hope: Our Fast Cure Plan

Gene therapy and systemic molecular research are our path to an affordable, full-body cure. Learn about our research →

Understanding Epidermolysis Bullosa