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Genetic and Rare Diseases Information Center (GARD)

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Epidermolysis bullosa acquisita


Other Names for this Disease
  • Acquired epidermolysis bullosa
  • EB acquisita
  • EBA
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Your Question

I have epidermolysis bullosa acquisita. Occasionally I have to have my fingernails removed, as they act like ingrown toe nails. My doctors tell me there is no other way to prevent them from growing back, except for phenol carbolic acid, and I had a reaction to that when they took off a toe nail. In addition, I would like to contact other people with epidermolysis bullosa acquisita.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is epidermolysis bullosa acquisita?

Epidermolysis bullosa acquisita (EBA) is a rare autoimmune disorder that causes the skin to blister in response to minor injury. Common areas of blistering include the hands, feet, knees, elbows, and buttocks. It can also affect the mouth, nose, and eyes. Some affected people have other health problems such as Crohn's disease, systemic lupus erythematosus, amyloidosis, or multiple myeloma. EBA is not inherited and usually occurs in adulthood. Treatment aims to protect the skin, stop the formation of blisters, and promote healing. Immunosuppressive drugs may be used to reduce the body's autoimmune response.[1]
Last updated: 2/27/2014

What causes epidermolysis bullosa acquisita?

The underlying cause of epidermolysis bullosa acquisita (EBA) is not known. It is thought to be an autoimmune disorder, which means that the immune system attacks healthy cells by mistake. In EBA, certain immune proteins (usually IgG autoantibodies) mistakenly target and attack a specific type of collagen (a skin protein) involved in "anchoring" the skin.[1][2] In some milder cases of EBA, the immune proteins involved are thought to be IgA, rather than IgG autoantibodies. The initiating event that leads to autoantibody production is unknown.[2]

EBA affecting several family members has been reported, suggesting a genetic component may be involved in some cases. Rarely, people with lupus, a systemic autoimmune disease, develop a generalized blistering skin disease with the features of EBA. EBA has also been associated with Crohn's disease.[2]
Last updated: 3/3/2014

Can epidermolysis bullosa acquisita affect fingernails and toenails?

Yes. Epidermolysis bullosa aquisita (EBA) may cause nail fragility, dystrophic (disordered growth of) finger nails, and nail loss.[3] We did not find information regarding targeted therapies for treatment of this symptom.
Last updated: 10/24/2011

How might epidermolysis bullosa acquisita be treated?

Due to the rarity of epidermolysis bullosa acquisita (EBA), data regarding the benefits of treatment is lacking. Treatments that have been tried, include systemic steroids, either alone or along with azathioprine, methotrexate, and cyclophosphamide, as well as intravenous immunoglobulin, rituximab and immunoadsorption.[4] To learn more regarding your treatment options, please speak with a healthcare provider.

Last updated: 10/24/2011

How can I connect with other people with epidermolysis bullosa acquisita?

Currently we are not aware of a support group or online community specifically for people with epidermolysis bullosa acquisita (EBA). However, you may still be able to connect with other's through the following online rare disease communities. If no one with EBA is currently registered, you can be the first to register, so that others can find you. 

The National Organization of Rare Disorders (NORD) has partnered with Inspire.com to launch an online community for people with rare diseases called The NORD Rare Disease Community. This community connects medical patients, family members, caregivers, and professionals. Click on The NORD Rare Disease Community to learn more.

Rare Disease Communities is an online social network for patients and families to connect with one another and share their experience living with a rare disease. The project is a joint collaboration between EURORDIS (European Rare Disease Organization) and NORD (National Organization for Rare Disorders). Click on the link above to view the community for Epidermolysis bullosa.

RareShare is an online social hub dedicated to patients, families and healthcare professionals affected by rare medical disorders. Click on RareShare to learn more.

Last updated: 10/24/2011

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