Epidermolysis bullosa acquisita
Other Names for this Disease
- Acquired epidermolysis bullosa
- EB 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?
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
- blisters on various mucous membranes
- significant scarring and dysfunction
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.
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." However, the condition is not thought to be due to any specific gene(s).
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. To learn more regarding your treatment options, please speak with a healthcare provider.
- Epidermolysis bullosa acquisita. DermNet NZ. December 29, 2013; http://www.dermnetnz.org/immune/epidermolysis-bullosa-acquisita.html. Accessed 2/27/2014.
- Gupta R, Woodley DT, Chen M. Epidermolysis bullosa acquisita. Clin Dermatol. January-February, 2012; 30(1):60-69. Accessed 3/3/2014.
- Jaggi Rao. Epidermolysis bullosa acquisita. Medscape Reference. April 5, 2013; http://emedicine.medscape.com/article/1063083-overview. Accessed 3/3/2014.
- 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.
- Gurcan HM, Ahmed AR . Current concepts in the treatment of epidermolysis bullosa acquisita. Expert Opin. Pharmacother. 2011;12(8):1259-1268;