Epidermolysis bullosa acquisita | |
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Other names | Acquired epidermolysis bullosa [1] |
Specialty | Dermatology |
Epidermolysis bullosa acquisita, also known as acquired epidermolysis bullosa, is a longterm autoimmune blistering skin disease. [1] It generally presents with fragile skin that blisters and becomes red with or without trauma. [2] Marked scarring is left with thin skin, milia and nail changes. [3] It typically begins around age 50. [2]
It is caused by antibodies to type VII collagen within anchoring fibril structures located at the dermoepidermal junction in skin. [3] Damaged skin may become infected. [3]
Diagnosis is by observing the persistence of the condition, direct immunofluorescence, and detecting autoantibodies against type VII collagen. [2] It can appear similar to porphyria cutanea tarda, pemphigoid, pemphigus, dermatitis herpetiformis, or blistering drug eruption. [3] The condition is longterm and has no cure. [1] A good response may be seen with corticosteroids, either alone or combined with azathioprine or dapsone. [3]
It is rare, with around 0.08 to 0.5 new cases per million people per year, and it affects males and females equally. [2]
It generally presents with fragile skin that blisters and becomes red with or without trauma. [2] Marked scarring is left with thin skin, milia and nail changes. [3] It typically begins around age 50. [2]
It is caused by antibodies to type VII collagen within anchoring fibril structures located at the dermoepidermal junction in skin. [3]
Diagnosis is by observing the persistence of the condition, direct immunofluorescence, and detecting autoantibodies against type VII collagen. [2] It can appear similar to porphyria cutanea tarda, pemphigoid, pemphigus, dermatitis herpetiformis, or blistering drug eruption. [3]
The condition is longterm and has no cure. [1] A good response may be seen with corticosteroids, either alone or combined with azathioprine or dapsone. [3]
It is rare, with around 0.08 to 0.5 new cases per million people per year, and it affects males and females equally. [2]