Vyjuvek

Generic name: beremagene-geperpavec-svdt
Dosage form: Topical gel
Drug class: Miscellaneous topical agents

Medically reviewed by Carmen Pope, BPharm. Last updated on May 23, 2023.

What is Vyjuvek?

Vyjuvek (beremagene-geperpavec-svdt) is a topical vector-based gene therapy that may be used to treat wounds in patients aged 6 months of age and older with dystrophic epidermolysis bullosa (DEB). It corrects the underlying skin defect of DEB by restoring functional copies of the COL7A1 gene to express COL7 and reassembling the anchoring fibrils that bind the dermis to the epidermis.

DEB is a serious, rare, genetic disorder that is one of four inherited forms of Epidermolysis Bullosa. It is caused by one or more mutations in the COL7A1 gene, resulting in the lack of production of functional type VII collagen (COL7) protein. The COL7 protein forms anchoring fibrils necessary to bind the dermis (inner layer of the skin) to the epidermis (outer layer of the skin). When there are no anchoring fibrils, the skin becomes extremely fragile, with minor friction or trauma (such as putting on a shirt) causing blisters and tears. Areas of the body primarily affected include the hands, feet, knees, and elbows.

Symptoms of DEB include open wounds, which lead to recurrent skin infections and fibrosis that can cause fusion of fingers and toes, and an increased risk of developing an aggressive form of squamous cell carcinoma (SCC).

Vyjuvek was approved on May 19, 2023, and is the first treatment for DEB to be approved by the FDA.

Warnings

There are no contraindications to the use of Vyjuvek.

How is Vyjuvek administered?

Vyjuvek is a topical gel that is applied by a healthcare provider to the wounds of patients with DEB once a week, either in the home or a healthcare setting. Once the gel is mixed with the biological suspension, the gel is dotted evenly over the wound in a grid-like pattern (1cm by 1cm apart) and then covered with a dressing.

The weekly dose that can be applied depends on the age of the patient.

• 6 months to <3 years: 1.6 x 10⁹ PFU or 0.8mL of reconstituted gel
• 3 years and older: 3.2 x 10⁹  PFU or 1.6mL of reconstituted gel.

The amount needed per wound depends on the size of the wound.

Vyjuvek can be re-dosed weekly as needed.

Related/similar drugs

Filsuvez

Patient information

Following treatment with Vyjuvek, patients or their caregivers should:

• Not directly touch the treated wound or the dressing covering it for approximately 24 hours
• Wear protective gloves when changing wound dressings and disposing of the dressings. Disinfect bandages from the first dressing change with an anti-viral agent and seal them in a plastic bag before putting them in the household waste. Put any subsequently used dressings in a sealed plastic bag in the household waste
• Flush any mucous membranes with clean water for at least 15 minutes in the event of accidental exposure.

For more information, patients, caregivers, and healthcare professionals can call Krystal Connect at 1-844-5-KRYSTAL.

What are the side effects of Vyjuvek?

Vyjuvek is well tolerated and common side effects include itching, chills, redness, rash, cough, and runny nose. In clinical trials, there were no drug-related serious adverse events or discontinuations due to treatment-related events.

To report suspected adverse reactions, contact Krystal Biotech, Inc. at 1-844-557-9782 or FDA at 1-800-FDA-1088 or http://www.fda.gov/medwatch.

How does Vyjuvek work?

Once applied to wounds the modified HSV-1 vector transduces both keratinocytes and fibroblasts and deposits the active ingredient of Vyjuvek, beremagene-geperpavec-svdt into the nucleus of these cells. This initiates the transcription of the encoded human COL7A1 and results in the production and secretion of mature COL7 which forms anchoring fibrils, essential for maintaining the integrity of the skin.

What are the ingredients in Vyjuvek?

Vyjuvek is a non-invasive, genetically modified, herpes simplex virus type 1 (HSV-1) vector-based, replication-deficient, nonintegrating gene therapy. HSV-1 represents a safe and straightforward way of delivering 2 copies of the COL7A1 gene without any adverse effects on human cells because modified HSV-1 will not replicate in the person’s cells nor integrate into the cell’s native genetic material.

Vyjuvek is supplied as a 1.0mL biological suspension in a single dose vial at a nominal concentration of 5×10⁹ PFU/mL (PFU = plaque forming units). The excipient gel is supplied as a 1.5 mL fill volume in a separate single-use vial. The suspension is mixed with the gel immediately before administration.

Once mixed, Vyjuvek gel consists of 5.0×10⁹ PFU in a volume of 2.5 mL. Neither the biological suspension nor the excipient gel contain preservatives

Manufacturer

Krystal Biotech, Inc.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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