Epidermolysis Bullosa Acquisita

What is Epidermolysis Bullosa Acquisita?

Epidermolysis bullosa acquisita, or EBA, is a rare blistering skin disease that often develops in adulthood. In fact, it usually occurs for the first time in one’s 40s or 50s, with men and women and all ethnicities being evenly affected. 

Some patients with EBA also suffer from other health issues like Crohn’s disease, lupus, amyloidosis, multiple myeloma, lung cancer and lymphoma. Other patients only have their skin affected.

 

Diagnosing EBA

We perform the following tests in order to properly diagnose you with epidermolysis bullosa acquisita:

  • Skin biopsy. This will show the presence of a blister under the epidermis as well as inflammatory cells in the dermis.

  • Direct immunofluorescence. This is done to shows an immune-mediated disease process. Autoantibody immunoglobulin G (IgG) is deposited in a line along the dermoepidermal junction.

  • Indirect immunofluorescence. This is a blood test to reveals the presence of circulating IgG autoantibodies that target the skin basement membrane and type VII collagen.

Other tests sometimes used to confirm the diagnosis include:

  • Skin biopsy for direct and indirect immunoelectron microscopy

  • Blood tests for immunoblotting, enzyme-linked immunosorbent assay (ELISA) and immunoprecipitation

 

Treatment

Because EBA is considered an autoimmune disease, immunosuppressive drugs to modify or reduce the autoimmune responses are often used as the primary treatment.

 

EBA is a chronic inflammatory disease that has periods of partial remissions and exacerbations. If treated and cared for properly, you can expect to live a normal lifespan.  

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