What Is EB and How Do Its Four Types Differ?

Exploring the 4 Types of EB: From Simplex to Kindler Syndrome

Imagine someone with extremely sensitive skin that is prone to blistering from any friction or contact. People with Epidermolysis Bullosa (EB) face a rare genetic skin disorder that causes the skin to become extremely fragile, leading to painful blisters and wounds from minor friction or trauma.

Affecting both external and internal body areas, EB presents serious complications and requires lifelong care. This blog explores the four main types of EB, Simplex, Junctional, Dystrophic, and Kindler Syndrome, along with other key details.

What is Epidermolysis Bullosa (EB)?

Epidermolysis Bullosa (EB) is a group of rare genetic conditions that cause inherited skin disorders, leading to fragile skin and blisters. Worldwide, EB-diagnosed prevalent cases reached 21,154 in 2024 and are expected to increase to 21,578 cases by 2029.

In this condition, sores, tears and blisters easily occur on the skin when facing small bumps or rubbing of the skin. Sore and painful blisters can appear anywhere, including on elbows, hands, feet and knees. These also occur inside the body, such as in the stomach, oesophagus, mouth, upper airway, bladder, intestines and genitals.

What are the 4 types of epidermolysis bullosa?

Doctors recognise the four types of EB depending on the location where skin changes or symptoms arise and the gene mutation. Here are the details:

1. Epidermolysis Bullosa Simplex (EBS): It is the common form of EB, which affects the lower part of the epidermis. In the mild range, blisters occur on the feet, palms and hands with or without scarring. However, in severe EBS, blistering spreads to the internal body areas, such as the throat or mouth.
2. Junctional Epidermolysis Bullosa (JEB): JEB is a severe genetic disorder that affects the upper basement membrane due to poor attachment between the epidermis and underlying layers. Blistering begins in infancy and can severely impact the mouth, vocal cords, and digestive tract, making eating painful and causing children to develop a hoarse, distressed cry over time.
3. Dystrophic Epidermolysis Bullosa (DEB): In mild DEB cases, doctors find blistering on the feet, knees, elbows, and hands. However, patients with severe DEB may experience poor nutrition and growth and blistering in the mouth and digestive system. People with severe dystrophic EB have higher chances of getting skin cancer over time.
4. Kindler Syndrome: In this condition, blistering can occur in the basement membrane and multiple layers of skin. Blisters are usually visible as early as infancy. Kindler syndrome causes thin, wrinkly and mottled skin and increases sensitivity to sunlight.

What are the symptoms of EB?

Symptoms of EB depend on the level of severity and the different types of EB. The common symptoms of all EBs include:

• Skin that easily blisters
• Fragile skin that is easily damaged, tears or scarring
• Blisters on the soles of the feet and hands
   

Symptoms of EB simplex include:

• Blisters in the mouth
• Thickened skin of the soles of the feet and palms of the hands
• Skin pigmentation changes
   

Severe junctional EB forms a serious health problem, and the signs are:

• Widespread blistering at birth
• Blisters in the upper airway, mouth, stomach, urinary tract, intestines, oesophagus, and genitals
• Absent toenails and fingernails
• Malnutrition
• Slow growth
• Anaemia
• Poor tooth enamel
   

Dystrophic EB symptoms depend on disease type, recessive or dominant:

• Blisters appear on the elbows, feet, and knees in the recessive subtype.
• Blistering inside the gastrointestinal tract and mouth in a severe condition
• Loss of nails
• Skin thickness, scarring
• Milia
• Slow growth
• Itching
• Tooth loss
• Eye damage in severe conditions
   

What Complications May Occur with EB?

Patients with EB can experience several complications that need to be spotted early. Complications may include:

• Bloodstream Infection
• Skin sensitivity to bacterial infections
• Change.
• Fusion fingers that cause unusual binding of joints
• A blister in the mouth creates difficulties in eating that lead to malnutrition.
• A painful blister in the anal area causes a constipation problem.
• Tooth decay
• Delay in wound healing
• Adults and teenagers may have skin cancer.
• Infants may face life-threatening complications including failure to thrive, severe infections, and respiratory compromise.
   

How to Treat Epidermolysis Bullosa?

Treatment of EB focuses on managing symptoms, preventing complications to support wound care, and reducing infections and pain, since there is no cure.

• A specific medicine, Oleogel-S10 gel, is used to heal wounds.
• Bandaging can heal wounds and protect skin from infection and injury.
• Occupational and physical therapy services prevent functional loss and weakness.
• Placing a feeding tube into the stomach can balance nutritional needs to support development and growth.
• Breathing tube placement through surgical treatment can reduce severe respiratory problems.
   

EB is a group of genetic skin disorders causing fragile skin, blistering, scarring, and nail thickening or loss. Treatment involves medication, bandaging, surgical repair to improve foot and hand function, wound care, and minimising pain.

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