FOR US RESIDENTS ONLY
NOW APPROVED For the treatment of Dystrophic Epidermolysis Bullosa (DEB) in patients 6 months and older

About Dystrophic
Epidermolysis Bullosa (DEB)

Cause of DEB

DEB is a type of epidermolysis bullosa (EB), a group of serious genetic blistering disorders

The most common symptom of DEB is fragile skin. This can lead to blisters, wounds, skin infections, and scarring.

  • There are 2 types of DEB: dominant and recessive
  • Whether you have dominant DEB (DDEB) or recessive DEB (RDEB), wounds can be large or small, take a long time to heal or not heal at all, and reopen after healing
  • Beyond symptoms you can see on the skin, DEB can affect other organs and body systems, including the eyes, mouth, gastrointestinal tract, and urinary tract
  • In addition to physical symptoms, people with DEB and those who care for them can face significant emotional, mental, and financial burdens
All DEB wounds should be taken seriously. It’s possible for any DEB wound to develop complications, such as infections or a certain type of aggressive skin cancer called squamous cell cancer (SCC).
How DEB Affects Skin

DEB is a genetic condition caused by variants in a gene called COL7A1

Genes carry instructions that tell the body how to build specific proteins. Changes in the instructions are called gene mutations or variants.

Skin not affected by DEB
  • The COL7A1 gene carries instructions to make a protein called type VII collagen (type 7 collagen)
  • Type VII collagen protein is the main component of anchoring fibrils. Anchoring fibrils help bind the inner skin layer (dermis) and outer skin layer (epidermis) together
Skin affected by DEB
  • In people with DEB, the variant in the COL7A1 gene means the body cannot create enough working type VII collagen protein
  • This leads to a lack of or reduced number of working anchoring fibrils. Without anchoring fibrils, the skin layers can easily separate due to friction
Illustrations adapted from De Rosa L., Latella MC, Secone Seconetti A, et al. Cold Spring Harb Perspect Biol. 2020.
Genetics of DEB

There are 2 types of DEB

DEB can be inherited in a dominant or recessive pattern.

People receive 2 versions of each gene, 1 from each parent. In dominant inheritance, only 1 copy of the gene with a mutation or variant is sufficient to cause a disorder. In recessive inheritance, 2 copies are required.

DDEB

Dominant dystrophic epidermolysis bullosa

DDEB occurs when a child inherits 1 copy of a dominant COL7A1 gene variant.

Dominant dystrophic epidermolysis bullosa (DDEB)

RDEB

Recessive dystrophic epidermolysis bullosa

RDEB occurs when a child inherits 2 copies of a recessive COL7A1 gene variant.

Recessive dystrophic epidermolysis bullosa (RDEB)
  • Unaffected
  • Carrier
  • Person with DEB
DDEB and RDEB symptoms range in severity, with similar symptoms and risks across both types.
Diagnosis

It’s important to accurately confirm DEB

DEB symptoms can appear the same as other EB types and even other skin conditions. This means diagnosis based on symptoms alone can be unreliable.

Tests to confirm DEB include:

DEB skin

Skin Biopsy can help show EB type, but it may be incomplete or inaccurate and may not be able to distinguish dominant DEB (DDEB) from recessive DEB (RDEB).

Genetic Testing

Genetic Testing uses a sample of blood or a cheek swab and can accurately identify DEB, as well as determine:

  • The specific COL7A1 gene mutation(s)
  • Whether it’s DDEB or RDEB
EB medical experts recommend genetic confirmatory testing to accurately diagnose DEB.
Learn About a Treatment for DEB
INDICATION AND USAGE

VYJUVEK is a topical gel used to treat wounds in patients 6 months and older with dystrophic epidermolysis bullosa (DEB).

IMPORTANT SAFETY INFORMATION

VYJUVEK gel must be applied by a healthcare provider.

After treatment, patients and caregivers should be careful not to touch treated wounds and dressings for 24 hours. If accidentally exposed to the VYJUVEK gel, clean the affected area.

Wash hands and wear protective gloves when changing wound dressings. Disinfect bandages from the first dressing change with a virucidal agent, and dispose of the disinfected bandages in a separate sealed plastic bag in household waste. Dispose of the subsequent used dressings in a sealed plastic bag in household waste.

Patients should avoid touching or scratching wound sites or wound dressings.

The most common adverse reactions (>5%) were itching, chills, redness, rash, cough, and runny nose. These are not all the possible side effects with VYJUVEK. Call your healthcare provider for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or to the Sponsor at 1-844-557-9782.

  • Please see Important Safety Information above and click here for full Prescribing Information.
IMPORTANT SAFETY INFORMATION
INDICATION AND USAGE

VYJUVEK is a topical gel used to treat wounds in patients 6 months and older with dystrophic epidermolysis bullosa (DEB).

IMPORTANT SAFETY INFORMATION

VYJUVEK gel must be applied by a healthcare provider.

After treatment, patients and caregivers should be careful not to touch treated wounds and dressings for 24 hours. If accidentally exposed to the VYJUVEK gel, clean the affected area.

Wash hands and wear protective gloves when changing wound dressings. Disinfect bandages from the first dressing change with a virucidal agent, and dispose of the disinfected bandages in a separate sealed plastic bag in household waste. Dispose of the subsequent used dressings in a sealed plastic bag in household waste.

Patients should avoid touching or scratching wound sites or wound dressings.

The most common adverse reactions (>5%) were itching, chills, redness, rash, cough, and runny nose. These are not all the possible side effects with VYJUVEK. Call your healthcare provider for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or to the Sponsor at 1-844-557-9782.

  • Please see Important Safety Information above and click here for full Prescribing Information.