| Epidermolysis bullosa acquisita | |
| Synonyms: | Acquired epidermolysis bullosa |
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]