epidermolysis bullosa

Last edited 10/2023 and last reviewed 10/2023

Epidermolysis bullosa

This is a rare group of mechano-bullous disorders in which a lack of cohesion between certain layers of the skin produces blistering in response to minor trauma (1).

A change in proteins which are responsible for maintaining skin integrity results in formation of bullae (2).

In mild varieties blistering may occur (mainly on hands and feet) only during the summer months in response to the friction of walking. In more severe varieties blistering may begin in utero and the child may be stillborn (2). The condition is inherited and genetic counselling is indicated.

Ccurrent therapy only focuses on the management of wounds and pain (3).

Birch bark extract is recommended, within its marketing authorisation, as an option for treating partial thickness wounds associated with dystrophic and junctional epidermolysis bullosa in people aged 6 months and over (4).

Major types include:

• junctional
• simplex
• dystrophic (2)

Click here for example images of this condition

Reference:

• 1. Das B.B, Sahoo S. Dystrophic Epidermolysis Bullosa. Journal of Perinatology 2004;24:41-47
• 2. Uitto J. Epidermolysis Bullosa: The Expanding Mutation Database. JID 2004;123
• 3. Prodinger C, Reichelt J, Bauer JW, Laimer M. Epidermolysis bullosa: Advances in research and treatment. Exp Dermatol. 2019 Oct;28(10):1176-1189.#
• 4.NICE (September 2023). Birch bark extract for treating epidermolysis bullosa