Complete Information on Epidermolysis bullosa acquisita with Treatment and Prevention

Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune subepidermal blistering disease of the rind and mucus membranes. It is a chronic incendiary disease with periods of incomplete remissions and exacerbations. Mortality as a direct consequence of the disease is rare; however, EBA is relatively unresponsive to treatment and can cause significant morbidity. All of the different types of epidermolysis bullosa are generally inherited. Therefore, having a family history of the disease, especially an affected parent, is a risk factor. The inheritance pattern may be dominant or recessive. A dominant form means that an offspring can inherit the gene or trait from one affected parent. Epidermolysis bullosa acquisita, however, usually appears in adults over age 50, although it has been reported in children.

Epidermolysis bullosa acquisita primarily involves the rind, but it too can impact mucus membranes. The trauma-prone areas of the rind, such as the extensor surfaces of elbows, knees, ankles, and buttocks, almost usually are affected. This may be indistinguishable from another autoimmune skin disorder called mucous membrane pemphigoid. Depending on the type present, epidermolysis bullosa varies in severity from minor blistering of the skin to a lethal form involving other organs. The condition generally starts at birth or soon after that. To prevent skin trauma and blistering, it may help to wear padding around trauma-prone areas like elbows, knees, ankles, and buttocks. Patients with epidermolysis bullosa acquisita who are on steroids for longer than one month may require calcium and vitamin D supplements to prevent osteoporosis.

Epidermolysis bullosa acquisita is characterized clinically by blisters, scars, and milia primarily at the trauma-prone areas. However, a subset of patients has a generalized incendiary rind blister phenotype. Most patients with epidermolysis bullosa acquisita experience a slow onset and chronic disease that affects the trauma-prone extensor skin surfaces. The nature of the disease usually leads to skin fragility, and the secondary scarring often causes restriction of mobility in the extensor skin surfaces. Some patients with EBA present with marked head and neck involvement, scarring, and minimal mucosal disease, which resembles the brunsting perry variant of cicatricial pemphigoid. For epidermolysis bullosa acquisita, there is an association with crohn's disease and, possibly, lupus. Symptoms of these conditions may be present, therefore, in adults who present with this type of epidermolysis bullosa.

The finish of handling is to forbid the establishment of blisters and consequent complications. The strength of maintenance depends upon the hardship of the disease. In addition, patients who are on long-term systemic corticosteroid treatment should take bisphosphonate, a class of specific inhibitor of osteoclast-mediated bone resorption. For patients who do not respond to oral corticosteroids and immunosuppressives, physicians could also consider using some other newer, but not-yet-proven therapeutic options, including intravenous immunoglobulin and intravenous infusion of anti-CD20 antibodies. Skin grafting for denuded or ulcerated areas of the skin may be necessary. Other surgical procedures for complications of epidermolysis bullosa might be recommended as well. Other treatments under investigation for epidermolysis bullosa include protein and gene therapy.