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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
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is epidermolysis bullosa acquisita?

What are the signs and symptoms of epidermolysis bullosa acquisita?

What causes epidermolysis bullosa acquisita?

Is epidermolysis bullosa acquisita inherited?

How might epidermolysis bullosa acquisita be treated?

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 are the signs and symptoms of epidermolysis bullosa acquisita?

Symptoms of epidermolysis bullosa acquisita (EBA) usually occur in a person's 30s or 40s. The signs and symptoms can differ among affected people, and the condition has several distinct forms of onset.[1] For example:

Non-inflammatory or mildly inflammatory EBA affecting only trauma-prone skin (the "classic" form) may cause:

  • tense, blood- or pus-filled blisters, mostly on the hands, knees, knuckles, elbows and ankles
  • mucous-membrane blisters that rupture easily
  • healing with significant scarring and small white spots (milia)

Generalized inflammatory EBA may cause:

  • widespread blisters that are not localized to trauma-prone sites
  • generalized redness and itching
  • healing with minimal scarring
The mucous membrane form of EBA may cause:
  • blisters on various mucous membranes
  • significant scarring and dysfunction[1]
The features of the condition may change during the course of the disease or may represent two forms at the same time.[2]
Last updated: 3/3/2014

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][3] 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.[3]

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.[3]
Last updated: 3/3/2014

Is epidermolysis bullosa acquisita inherited?

Unlike the genetic forms of epidermolysis bullosa, epidermolysis bullosa acquisita (EBA) is considered an acquired, sporadic disease. This means that it generally occurs in people with no history of the condition in their families.

There have been a couple of reports of families with more than one affected person, suggesting a genetic component may be involved. This could mean that EBA may develop in a person who is "genetically susceptible."[4] However, the condition is not thought to be due to any specific gene(s).
Last updated: 3/3/2014

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.[5] To learn more regarding your treatment options, please speak with a healthcare provider.

Last updated: 10/24/2011

References
  1. Epidermolysis bullosa acquisita. DermNet NZ. December 29, 2013; http://www.dermnetnz.org/immune/epidermolysis-bullosa-acquisita.html. Accessed 2/27/2014.
  2. Gupta R, Woodley DT, Chen M. Epidermolysis bullosa acquisita. Clin Dermatol. January-February, 2012; 30(1):60-69. Accessed 3/3/2014.
  3. Jaggi Rao. Epidermolysis bullosa acquisita. Medscape Reference. April 5, 2013; http://emedicine.medscape.com/article/1063083-overview. Accessed 3/3/2014.
  4. Noe MH, Chen M, Woodley DT, Fairley JA. Familial epidermolysis bullosa acquisita. Dermatol Online J. December 15, 2008; 14(12):2. Accessed 3/3/2014.
  5. Gurcan HM, Ahmed AR . Current concepts in the treatment of epidermolysis bullosa acquisita. Expert Opin. Pharmacother. 2011;12(8):1259-1268;


Other Names for this Disease
  • Acquired epidermolysis bullosa
  • EB acquisita
  • EBA
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.