Epidermolysis bullosa acquisita (EBA) is a rare autoimmune disorder that causes the skin to blister in response to minor injury. Common areas of blistering include the hands, feet, knees, elbows, and buttocks. It can also affect the mouth, nose, and eyes. Some affected people have other health problems such as Crohn's disease, systemic lupus erythematosus, amyloidosis, or multiple myeloma. EBA is not inherited and usually occurs in adulthood. Treatment aims to protect the skin, stop the formation of blisters, and promote healing. Immunosuppressive drugs may be used to reduce the body's autoimmune response.

EBA affecting several family members has been reported, suggesting a genetic component may be involved in some cases. Rarely, people with lupus, a systemic autoimmune disease, develop a generalized blistering skin disease with the features of EBA. EBA has also been associated with Crohn's disease.

There have been a couple of reports of families with more than one affected person, suggesting a genetic component may be involved. This could mean that EBA may develop in a person who is "genetically susceptible." However, the condition is not thought to be due to any specific gene(s).

Non-inflammatory or mildly inflammatory EBA affecting only trauma-prone skin (the "classic" form) may cause:

• tense, blood- or pus-filled blisters, mostly on the hands, knees, knuckles, elbows and ankles
• mucous-membrane blisters that rupture easily
• healing with significant scarring and small white spots (milia)

Generalized inflammatory EBA may cause:

• widespread blisters that are not localized to trauma-prone sites
• generalized redness and itching
• healing with minimal scarring

The mucous membrane form of EBA may cause:

• blisters on various mucous membranes
• significant scarring and dysfunction

The features of the condition may change during the course of the disease or may represent two forms at the same time.

Treatment of EBA may require a multidisciplinary team of doctors. Affected individuals with involvement of the gastrointestinal tract should be referred to a gastroenterologist. Likewise, dentists and ophthalmologists should be consulted for those with oral and ocular (eye) involvement. Medications used to treat EBA include oral corticosteroids (such as prednisone), anti-inflammatory agents (such as dapsone or colchicine), and medications that suppress the immune system (such as azathioprine or cyclophosphamide). Patients who are on long-term oral steroid treatment may be advised to take calcium, vitamin D, and, perhaps, bisphosphonates to reduce the risk of osteoporosis that can be associated with steroid use. Extracorporeal photochemotherapy (photophoresis) has been shown to be effective in some cases. In patients who do not respond to other therapies, medications such as rituximab may be considered. Patients with EBA should avoid contact sports and direct physical trauma to the skin whenever possible and may be instructed to use protective pads to cover skin surfaces when undergoing planned physical activities. Patients with oral involvement should avoid hard foods such as pretzels, nuts, and chips. Patients with EBA should be monitored regularly by physicians with experience in treating autoimmune skin disease. During the active disease stage, patients should be monitored on a monthly basis; when in remission, patients should be monitored annually.