Epidermolysis Bullosa Acquisita Case Study

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Epidermolysis Bullosa Acquisita: The Rare, Acquired, Chronic
Disease of Blister Formation Epidermolysis Bullosa Acquisita (EBA) is a rare, acquired, autoimmune disease that produces the formation of blisters on the skin after minor trauma (“Epidermolysis Bullosa Clinic”, 2015). Due to small rubs and strains, blisters are commonly seen on the skin. EBA does not only affect the skin, but it can also affect the eyes, nails, mouth, esophagus, and lungs. (Prabhu, Ramesh, Rekka, Swathi, 2011). EBA has a range of mild to life threatening symptoms (Prabhu, et al., 2011). At its mildest state, EBA is confined to the hands and feet making it very difficult and painful to hold objects and walk (Prabhu et al., 2011). In more severe cases, all of the
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The skin consists of two layers: the outer layer, epidermis and the inner, dermis. In healthy skin, the epidermis and dermis are anchored and held together by a specific protein that prevents the two layers from shearing and moving freely from one another (Schmidt & Zillikens, 2013). In cases of EBA births, the two layers of skin are not anchored together resulting in fragile skin. (Schmidt & Zillikens, 2013). Type VII collagen (COL7) is a protein located in the basement membrane of the skin and is an autoantibody of EBA patients (Aoyama, Iwata, Kamio, 2010). Though, it was discovered through mice injections that the sole binding of the antibody did not create skin blistering or deformity. Several other cellular components are needed to produce the EBA phenotype, which include: neutrophils, Fc factors, and complement activation (Ludwig, 2012). EBA pathogenesis and autoantibody- induced cellular damage can be understood as: (i) binding of autoantibody to antigen, (ii) Fc- factors of blister production, and (iii) the diapedesis of neutrophils (Ludwig, …show more content…
Blistering around the eyes and nose and blistering around the mouth and esophagus cause feeding difficulties and dysphagia (Krol, Lofgren, 2011). Tooth decay, cough and other breathing problems, alopecia, milia, and nail loss are other symptoms of Epidermolysis Bullosa Acquisita (Krol, Lofgren, 2011). EBA may not occur until the toddler age when they begin to walk and partake in more physical activities that trigger friction (Aoyama et al., 2011).
Treatment of EBA Many people with mild Epidermolysis Bullosa Acquisita require little to no treatment; however, patients with more severe forms of EBA require daily intensive care and treatment (“Epidermolysis Bullosa Clinic”, 2015). There is no cure for EBA, but current treatments of severe EBA focus on the use of cyclosporine, intravenous immunoglobulin (IVIG), and wound healing and protection of the skin (“Epidermolysis Bullosa Clinic”, 2015).
Cyclosporine is an immunosuppressant drug that slows the immune system to decrease further damage of the skin (“Cyclosporine”). Cyclosporine is effective in the majority of EBA patients (Ludwig,

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