Epidermolysis bullosa acquisita
Epidermolysis bullosa acquisita | |
---|---|
Synonyms | EBA |
Pronounce | N/A |
Specialty | N/A |
Symptoms | Blister formation, skin fragility, scarring, mucosal involvement |
Complications | Infection, scarring, esophageal strictures |
Onset | Usually adulthood |
Duration | Chronic |
Types | N/A |
Causes | Autoimmune disease |
Risks | Genetic predisposition, trauma, inflammation |
Diagnosis | Skin biopsy, immunofluorescence |
Differential diagnosis | Bullous pemphigoid, epidermolysis bullosa, pemphigus vulgaris |
Prevention | N/A |
Treatment | Corticosteroids, immunosuppressants, plasmapheresis |
Medication | Dapsone, colchicine, rituximab |
Prognosis | Variable, depends on severity and response to treatment |
Frequency | Rare |
Deaths | N/A |
Epidermolysis bullosa acquisita (EBA) is a rare autoimmune disease that causes the skin to blister and scar. It is a form of epidermolysis bullosa, a group of genetic conditions that cause the skin to be very fragile and to blister easily.
Symptoms
The main symptom of EBA is the formation of blisters on the skin. These blisters can form anywhere on the body, but are most common on the hands, feet, knees, and elbows. The blisters can be painful and may leave scars when they heal. Other symptoms can include itching, redness, and swelling of the skin.
Causes
EBA is caused by the body's immune system attacking the skin. This is why it is classified as an autoimmune disease. The immune system mistakenly targets a protein in the skin called type VII collagen, which is important for the structure and strength of the skin.
Diagnosis
Diagnosis of EBA can be challenging, as its symptoms can be similar to those of other skin conditions. Doctors may use a variety of tests to diagnose EBA, including blood tests, skin biopsy, and immunofluorescence testing.
Treatment
There is currently no cure for EBA, but treatments can help manage the symptoms. These may include topical steroids, immunosuppressive drugs, and physical therapy to help improve mobility if the blisters and scars have affected the joints.
See also
References
External links
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