Title: Oleogel-S10 for treating skin wounds associated with epidermolysis bullosa [ID1505]
Funder: Department of Health and Social Care

Abstract: Epidermolysis bullosa (EB) is a general term used to describe a group of rare inherited skin disorders that cause the skin to become very fragile. Any trauma or friction can cause the skin to blister and tear easily. There are different types of EB, and the condition is classified according to where on the body the blistering takes place and which layer of skin is affected[1] . Symptoms can vary significantly by subgroup: _x000D_ _x000D_ • dystrophic epidermolysis bullosa (DEB) – accounts for around 25%[2] of cases and can be either dominantly or recessively inherited. Dominant DEB is the mildest form of DEB, with recessive DEB associated with more severe symptoms. Blistering occurs below the basement membrane zone in the upper part of the dermis (lower layer of the skin). In mild forms blistering is limited to the hands, feet, knees, and elbows, but may be widespread in more severe cases. _x000D_ _x000D_ • junctional epidermolysis bullosa (JEB) – the rarest and most severe type is classified as either ‘generalised severe’ (Herlitz) or ‘generalised intermediate’ (non-Herlitz). In the severe form blistering can cover large regions of the body including the lining of the mouth and digestive tract. Around 40% of children born with generalized severe (Herlitz) JEB, the more severe form of JEB, will not live past the first year and most won’t survive five years[1] . _x000D_ _x000D_ • epidermolysis bullosa simplex (EBS) – accounts for 70%[2] of cases and tends to be milder, although blisters can occur anywhere on the body they are often confined to the palms and soles. EBS has a low risk of serious complications. _x000D_ _x000D_ • Kindler Syndrome is also a rare type of EB which is characterised by blisters which are formed in different layers of the skin and symptoms such as pigmentation and photosensitivity. _x000D_ _x000D_ As well as external blisters, EB can manifest internally affecting areas such as the eye, mouth or stomach. Other complications associated with EB can include the development of aggressive skin cancers, dental problems, or nutritional compromise. _x000D_ _x000D_ EB is usually diagnosed in babies and children and is thought to affect 1 in 17,000 births with around 5,000 people affected in the UK[3] . _x000D_ _x000D_ There is currently no cure for EB. Treatments help ease and control symptoms. It aims to avoid skin damage, improve quality of life and reduce the risk of developing complications such as infection and malnutrition[1] . Given the complex needs of children with EB, treatment is usually carried out by a multidisciplinary team._x000D_ _x000D_ References_x000D_ 1. NHS choices. Epidermolysis bullosa. Available at https://www.nhs.uk/conditions/epidermolysis-bullosa/ (assessed 12th March 2020)_x000D_ 2. DEBRA. Epidermolysis bullosa/ Available at https://www.debra.org.uk/types-ofeb/intro (accessed 12th March)_x000D_ 3. Mellerio JE; Epidermolysis bullosa care in the United Kingdom. Dermatol Clin. 2010 Apr28(2):395-6, xiv. doi: 10.1016/j.det.2010.02.015.

Lay abstract: To appraise the clinical and cost effectiveness of birch bark extract within its marketing authorisation for treating skin wounds associated with dystrophic and junctional epidermolysis bullosa.

Research activity codes: 6.1 PHARMACEUTICALS
Health categories: SKIN