Kindler EB - Debra Ireland

Kindler EB

Kindler EB (KEB), previously known as Kindler Syndrome, is a rare form of EB. Blistering can occur in all layers of the skin or internal organs but tends to affect the extremities.

About Kindler EB (KEB)

What causes Kindler EB (DEB)?

Every person has two copies of each gene, the gene mutation that causes EB can be in one or both genes in a pair. However, Kindler EB is inherited recessively meaning that both genes in a pair – one from each parent – is affected.

Recessive EB is usually more severe than dominant types and can come as a complete shock as the parents can be carriers without displaying symptoms themselves. The chances of a child inheriting Kindler EB is 25%.

Source: DEBRA International

What are the symptoms of KEB?

  • Blistering can affect the whole body including internal organs but tends to be most severe on hands, feet and moist linings such as the eyes, intestines, oesophagus, mouth, urinary tract and genitals.
  • There is often a high sensitivity to bright light, meaning the skin can burn easily in the sun.
  • Discolouration of the skin and hyperkeratosis (thickening of skin) can occur on the feet and palms.
  • Gingivitis (gum disease) is common and blistering in the mouth can make eating uncomfortable.
  • Inflammation of the intestines is also common, which can affect digestion.
  • There is an increased risk of non-melanoma skin cancers.


There is currently no cure for EB, our work aims to change this. However, there are treatments available which help with the management of pain and itch.

We fund research projects aiming to find additional treatments as well as a cure, and our EB Support Team are committed to help people and carers deal with the challenges that EB brings.

How can we help you?

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