WHAT IS EB?
EB is a group of rare genetic disorders characterised by fragility of the skin and mucous membranes, and mechanically induced blistering. EB comprises four main types - EB simplex (EBS), junctional EB (JEB), dystrophic EB (DEB), and Kindler EB (KEB), with more than 30 subtypes. EB is clinically heterogeneous including a broad spectrum of severity.
Classification of EB into four main types is based on where splitting occurs in the different layers of skin. Reclassification of the condition takes place when new genes and clinical subtypes have been identified.
In EB simplex (EBS), splitting occurs within the upper layer of the skin, the epidermis.
In junctional EB (JEB), splitting occurs within the structure that keeps the epidermis and the underlying dermis together, the basement membrane.
In dystrophic EB (DEB), splitting occurs below the basement membrane within the superficial dermis.
In Kindler EB (KEB), blistering can occur at multiple and different levels within the skin.