As a result of epidermolysis bullosa acquisita (EBA), blisters and erosions appear on the skin and mucous membranes. In this article, we examine the causes, symptoms, diagnosis, treatment, and prevention of epidermolysis bullosa acquisita.
Causes
A type VII collagen protein that anchors the layers of the skin together plays a crucial role in the development of epidermolysis bullosa acquisita because it is produced by autoantibodies. As a result of these autoantibodies attacking the anchoring fibrils, blisters and skin fragility result, leading to the separation of the epidermis from the underlying dermis.
Symptoms
As a primary symptom of epidermolysis bullosa acquisita, blisters and erosions occur on the skin and mucous membranes, particularly in areas prone to friction or trauma, such as the hands, feet, knees, elbows, and mouth. Over time, these blisters can cause scarring and skin deformities, and they can result in nail dystrophy, joint contractures, and difficulty swallowing if mucous membranes are affected.
Diagnosis
Clinical evaluation, skin biopsy, and laboratory tests are used to diagnose epidermolysis bullosa acquisita. The presence of characteristic changes, including subepidermal blistering and inflammatory infiltrates, is assessed under a microscope. As an alternative to immunofluorescence, IgG antibodies can also be detected along the basement membrane zone through immunofluorescence studies. In addition, blood tests can be conducted to measure the level of autoantibodies against type VII collagen.
Treatment
Epidermolysis bullosa acquisita is treated by reducing blistering, managing symptoms, and preventing complications.
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Blisters and erosions can be treated with topical steroids to reduce inflammation and promote healing.
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Oral or intravenous immunosuppressive medications, such as corticosteroids, methotrexate, or cyclosporine, can suppress the immune system and reduce autoantibody production.
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A biologic agent, such as rituximab or intravenous immunoglobulin (IVIG), may be used to target specific components of the immune system involved in the pathogenesis of EBA.
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Using non-adherent dressings and protective padding can help prevent secondary infections and promote healing of wounds.
Prevention
Since epidermolysis bullosa acquisita is an autoimmune disorder, there are no specific preventive measures to avoid its onset. However, early diagnosis and prompt treatment can help manage symptoms and improve outcomes. In addition, individuals with EBA may benefit from avoiding trauma or friction to their skin and mucous membranes.
Epidermolysis bullosa acquisita is a rare autoimmune disorder characterized by blisters and erosions of the skin and mucous membranes caused by autoantibodies against type VII collagen. For individuals who are affected by this condition to be effectively managed and have a better quality of life, it is essential to understand its causes, symptoms, diagnosis, treatment, and preventive strategies.
