Epidermolysis bullosa acquisita

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. 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.

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.

Last updated on 05-01-20

Can epidermolysis bullosa acquisita cause dizziness?

To our knowledge, epidermolysis bullosa acquisita is not associated with dizziness.

Last updated on 05-01-20

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." However, the condition is not thought to be due to any specific gene(s).

Last updated on 05-01-20

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. We did not find information regarding targeted therapies for treatment of this symptom.

Last updated on 05-01-20

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 on 05-01-20

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.

Last updated on 05-01-20

How might epidermolysis bullosa acquisita be treated?

Treatment of EBA may require a multidisciplinary team of doctors. Affected individuals with involvement of the gastrointestinal tract should be referred to a gastroenterologist. Likewise, dentists and ophthalmologists should be consulted for those with oral and ocular (eye) involvement. Medications used to treat EBA include oral corticosteroids (such as prednisone), anti- inflammatory agents (such as dapsone or colchicine), and medications that suppress the immune system (such as azathioprine or cyclophosphamide). Patients who are on long-term oral steroid treatment may be advised to take calcium, vitamin D, and, perhaps, bisphosphonates to reduce the risk of osteoporosis that can be associated with steroid use. Extracorporeal photochemotherapy (photophoresis) has been shown to be effective in some cases. In patients who do not respond to other therapies, medications such as rituximab may be considered. Patients with EBA should avoid contact sports and direct physical trauma to the skin whenever possible and may be instructed to use protective pads to cover skin surfaces when undergoing planned physical activities. Patients with oral involvement should avoid hard foods such as pretzels, nuts, and chips. Patients with EBA should be monitored regularly by physicians with experience in treating autoimmune skin disease. During the active disease stage, patients should be monitored on a monthly basis; when in remission, patients should be monitored annually.

Last updated on 05-01-20

Clinical Research Resources

Completed clinical trial - Epidermolysis bullosa acquisita

The National Center for Research Resources (NCRR) in cooperation with Northwestern University has completed a clinical trial entitled 'Phase II Pilot Study of Extracorporeal Phototherapy for Epidermolysis Bullosa Acquisita' (study ID: 199/11928, NU-511; NLM Identifier NCT00004359). To view the study abstract click on the link above.

To locate information resulting from the completed study, you can either e-mail or call the National Library of Medicine (NLM) Customer Service. Include the title of the study, the study ID number, and the NLM Identifier, and a librarian at NLM can assist you in searching the medical literature for published results on the completed clinical trial.

National Library of Medicine Customer Service
Toll-free: 888-346-3656
E-mail: custserv@nlm.nih.gov

Last updated on 04-27-20

Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States
Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: aarda@aarda.org Url: https://www.aarda.org/

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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