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EB Acquisita (EBA) & Kindler EB

EB Acquisita (EBA) & Kindler EB

 

EB Acquisita (EBA)

EB Acquisita (EBA) is the rarest type of Epidermolysis Bullosa (EB), a painful genetic condition causing the skin to tear or blister at the slightest touch. Most types of EB are caused by gene mutations which affect different layers of the skin and sometimes internal organs. However, EB Acquisita is classified as an autoimmune disease whereby the body starts to attack its own healthy body tissue.

 

What causes EBA?

EBA is the only type of EB not to be hereditary. It is extremely rare and the specific cause is not known but it does tend to develop in adulthood. It is thought that immune proteins mistakenly attack healthy collagen – the skin protein which binds the skin together.

 

Symptoms of EBA

EBA is very rare and is usually mild to moderate. Blistering tends to affect the hands, knees, knuckles, elbows and ankles the most. The prognosis is dependent on the whether there is any underlying problem but a normal life expectancy is possible and a range of treatments  are available to help with symptoms. EBA tends to be more common in people with other autoimmune diseases, such as Crohn’s and Lupus.

Researchers have identified 3 types of EBA, broadly categorised by Inflammatory and non-inflammatory types:

  • Classic or Non-Inflammatory EBA - causing skin blistering mostly on the hands, knees, knuckles, elbows, ankles and mucous membrane areas (an area of the body lined with a membrane such as mouth, throat, eyes & stomach). Scarring can occur or white spots (milia) form.
  • Generalized Inflammatory EBA – widespread blistering, redness and itching, healing with minimal scarring.
  • Mucous Membrane Inflammatory EBA - blistering of the mucous membrane with possible significant scarring.

 

Treatments

There is currently no cure for EB, our work at DEBRA aims to change this. With EBA, treatment is mostly focused on the use of immunosuppressive medication. 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 Community Support Team are always here to help patients and families deal with the challenges that EB brings.

 

Support

If you or your family member have been diagnosed with EB Acquisita, you can contact our Community Support Team for additional support. Our team aim to support the whole EB community regardless of the type or severity, we have a range of practical, emotional and financial support options. Find out more about how we can help people living with EB here

 

Kindler EB

Kindler EB is one of five main types of Epidermolysis Bullosa (EB), a painful genetic condition causing the skin to tear or blister at the slightest touch. Most types of EB are caused by gene mutations which affect different layers of the skin and sometimes internal organs.  

Kindler EB is the rarest type of inherited EB - there is one extremely rare type of EB, EB Acquisita, which is classified as an autoimmune disease and is not inherited. With Kindler EB, blistering can occur in all layers of the skin or internal organs but has a tendency to affect the extremities.


What causes Kindler EB?

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%.

Find out more about the difference between dominant and recessive EB here

 

Symptoms of Kindler EB

Kindler EB (previously Kindler Syndrome), although very rare, can be severe but often a normal life expectancy is possible and a range of treatments are available to help with symptoms:

  • 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.   
     

Treatments

There is currently no cure for EB, our work at DEBRA UK aims to change this. However, there are treatments available which help with the management of pain and itch. If you are living with EB speak to your healthcare provider about treatment options. We fund research projects aiming to find additional treatments as well as a cure, and our Community Support Team are always here to help patients and families deal with the challenges that EB brings.

 

Support

If you or your family member have been diagnosed with Kindler EB, you can contact our Community Support Team for additional support. Our team aim to support the whole EB community regardless of the type or severity, we have a range of practical, emotional and financial support options. Find out more about how we can help people living with EB here

 

Find out more about other types of EB