Epidermolysis Bullosa Acquisita (EBA) is a rare autoimmune skin disorder characterized by the formation of blisters and erosions on the skin and mucous membranes. It is a subtype of the broader Epidermolysis Bullosa (EB) group of disorders, which are characterized by skin fragility and blistering.
In EBA, the immune system mistakenly targets and attacks the anchoring fibers that hold the layers of the skin together. This leads to the separation of the epidermis (outermost layer of the skin) from the underlying dermis, resulting in painful, fluid-filled blisters that are prone to rupture and form erosions or ulcers. The blisters commonly occur on the hands, feet, knees, elbows, and buttocks. Mucous membranes such as the mouth, eyes, and genitals may also be affected.
EBA typically starts in adulthood, often between the ages of 30 and 50. Its exact cause is unknown, although it is believed to be an autoimmune disorder where the body's immune system mistakenly targets its own tissues. EBA can be associated with other autoimmune conditions such as systemic lupus erythematosus and inflammatory bowel disease.
Currently, there is no cure for EBA, and management focuses on relieving symptoms and preventing complications. Treatment may involve wound care, infection control, pain management, and the use of medications that suppress the immune system. Advanced treatments like skin grafting or gene therapy may be considered in severe cases.
Given its chronic nature, EBA significantly impacts an individual's quality of life and requires long-term management and support from a multidisciplinary healthcare team.
