Epidermolysis bullosa acquisitaЖ Description, Causes and Risk Factors:Abbreviation: EBA.Epidermolysis bullosa acquisita is an acquired autoimmune bullous disease characterized by tense blisters arising on inflamed or normal appearing skin at trauma-exposed sites. In EBA the body attacks its own anchoring fibrils (collagen fibrils that insert in to the basal lamina of the epidermis and bind it down to the underlying dermis) with antibodies, the special proteins that help fight and destroy foreign substances that invade the body. In a few cases, it has occurred following drug therapy for another condition; in most cases, the cause is unknown.Most people with Epidermolysis bullosa acquisita have inherited the condition through faulty genes they receive from one or both parents. Genes are located in the body's cells and determine inherited traits passed from parent to child. They also govern every body function, such as the formation of proteins in the skin. More than 10 genes are known to underlie the different forms of Epidermolysis bullosa acquisita. Genes are located on chromosomes, which are structures in each cell's nucleus.Most Epidermolysis bullosa acquisita patients have IgG antibodies that recognize the non-collagenous domain of collagen VII, although there is a subset of patients with IgG that targets the collagenous domain. There is also a subset of patients with IgA, rather than IgG autoantibodies. Evidence for a causative relationship between the presence of collagen VII antibodies and blister formation comes from the observation that passive transfer of antibodies from Epidermolysis bullosa acquisita patients to mice is able to cause blister formation in areas of trauma in the mouse. The fact that blister formation does not occur in mice lacking proper complement activation ability suggests that the mechanism of EBA blister formation is complement mediated. Complement deposition is observed along with immunoglobulins at the basement membrane in Epidermolysis bullosa acquisita patients.It is linked to Crohn's disease (an inflammatory bowel disease) and possibly lupus. Adults with this type of epidermolysis bullosa may also have symptoms of these other conditions.Two distinct phenotypes of Epidermolysis bullosa acquisita have been described: non-inflammatory (classical) EBA and inflammatory EBA. Both types may present as indicated above, however, inflammatory EBA can also mimic other subepidermal bullous disorders such as bullous pemphigoid, mucous membrane pemphigoid, and linear IgA dermatosis. Most EBA occurring in adults is classical EBA, while EBA in childhood is rare and usually of the inflammatory type. One feature that distinguishes presentation of adult Epidermolysis bullosa acquisita from the childhood form is mucosal involvement. In adult EBA mucosal involvement occurs in approximately 50% of cases as compared with childhood EBA in which mucosal involvement occurs in the majority of cases.There is an estimated annual incidence of 0·22 per million population in Western Europe. It tends to affect people over 40 years of age, although children may also be affected. Like cicatricial pemphigoid, there is a slight female preponderance.Symptoms:The major sign of all forms of EB is fragile skin that blisters,which can lead to serious complications. For example,blistering areas may become infected, and blisters in themouth or parts of the gastrointestinal tract may interfere withproper nutrition.Other symptoms may include:Alopecia (hair loss).
Blisters around the eyes and nose.
Blisters in or around the mouth and throat, causing feeding problems or swallowing difficulty.
Blisters on the skin as a result of minor injury or temperature change.
Blistering that is present at birth.
Dental problems such as tooth decay.
Hoarse cry, cough, or other breathing problems.
Tiny white bumps or pimples (milia).
Nail loss or deformed nails.
Diagnosis:Differential diagnosis may include:Pemphigoid.
To establish the diagnosis of Epidermolysis bullosa acquisita, thefollowing tests should be performed:H &E biopsy taken from the edge ofa new blister.
Direct immunofluorescence onnormal-appearing perilesional skin.
Indirect immunofluorescence usingthe patient's serum on a salt-splitnormal skin substrate.
Upper endoscopy or an upper GI series if there are swallowing or feeding difficulties.
Treatment:The goal of treatment is to prevent blisters from forming and complications. How much treatment is needed depends on how severe the disease is. Recommendations often include avoiding skin damage (trauma) and hot environments.Classical Epidermolysis bullosa acquisita may be refractory to many treatments such as to corticosteroids, methotrexate and cyclophosphamide. The inflammatory type of EBA responds more favorably to treatment with dapsone and prednisone. Other agents such as cyclosporine in high dose, IV immunoglobulins, and plasmapheresis may also be of help. Supportive therapy is of utmost importance in EBA.Preventive Measures:To prevent infection take very good care of the skin, especially if any blistered areas become crusted or exposed (raw). Follow your health care provider's instructions closely. You might need regular whirlpool therapy and to apply antibiotic ointments to wound-like areas. Your health care provider will let you know if you need a bandage or dressing, and if so, what type to use.
Proper nutrition is also important. When you have a lot of skin injury, you may need extra calories and protein to help you recover. Work closely with a nutritionist. If you have blisters or complications in the mouth or esophagus, avoid eating hard or brittle foods such as pretzels, nuts, and chips. Eating soft foods can help prevent making the sores worse.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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