Epidermolysis Bullosa Aquisita (EBA)
Epidermolysis Bullosa Aquisita (EBA) is a rare autoimmune disease characterized by sub-epithelial blistering of the skin and mucosal membranes in response to injury. Patients with EBA suffer from chronic inflammation as well as blistering and scarring of the skin and mucous membranes. This mostly occurs in the hands, feet, knees, elbows, buttocks, mouth, nose, and eyes. EBA is not inherited and usually occurs in adulthood, it usually emerges mostly when patients are in their 40s or 50s, but EBA can occur at any age.
Some affected patients have other health problems such as Amyloidosis, Bullous Pemphigoid, Crohn’s disease, Systemic Lupus Erythematosus and Multiple Myeloma. 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. Current treatment options rely on non-specific immunosuppression, which in many cases, does not lead to a remission of treatment.
Symptoms & Types of Epidermolysis Acquisita (EBA)
Signs and Symptoms of Epidermolysis Bullosa Acquisita (EBA) can differ among affected patients, and the condition has several distinct forms of onset. The features of the condition in EBA patients may change during the course of the disease or may represent two forms at the same time, For example:
Non-inflammatory or mildly inflammatory Epidermolysis Bullosa Acquisita (EBA)
Non-inflammatory or mildly inflammatory Epidermolysis Bullosa Acquisita (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 Epidermolysis Bullosa Acquisita (EBA)
Generalized inflammatory Epidermolysis Bullosa Acquisita (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 Epidermolysis Bullosa Acquisita (EBA)
- Blisters on various mucous membranes
- Significant scarring and dysfunction
What Causes Epidermolysis Bullosa Acquisita
The underlying causes of Epidermolysis Bullosa Acquisita (EBA) are unknown. 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 events that leads to autoantibody production are unknown.
Detailed clinical data, elevated levels of antibodies, serology, biomarkers, CD-20, Type VII collagen, genetic info, skin biopsy tissue, pathology annotations, associated with the Epidermolysis Bullosa Aquisita patient’s specimens is provided to a valued customer for drug discovery, development and research. The Epidermolysis Bullosa Aquisita disease sera (serum), plasma and PBMC biofluid samples were processed from patients peripheral whole blood using customized processing protocols provided by the researcher.
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