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

• Autoimmune disease
• Epidermolysis bullosa
• Blister
• Type VII collagen

References

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External links

• Epidermolysis bullosa acquisita at PubMed Health
• Epidermolysis bullosa acquisita at National Organization for Rare Disorders

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