Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

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.

Cause

Newline Maker

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

References
  1. Epidermolysis bullosa acquisita. DermNet NZ. December 29, 2013; http://www.dermnetnz.org/immune/epidermolysis-bullosa-acquisita.html. Accessed 2/27/2014.
  2. Jaggi Rao. Epidermolysis bullosa acquisita. Medscape Reference. April 5, 2013; http://emedicine.medscape.com/article/1063083-overview. Accessed 3/3/2014.


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.