期刊
AUTOIMMUNITY
卷 45, 期 1, 页码 91-101出版社
TAYLOR & FRANCIS LTD
DOI: 10.3109/08916934.2011.606450
关键词
Epidermolysis bullosa acquisita; type VII collagen; skin; anchoring fibrils; autoimmunity
类别
资金
- National Institutes of Health [RO1 AR47981, RO1 AR33625]
Epidermolysis bullosa acquisita (EBA) is a rare and acquired autoimmune subepidermal bullous disease of skin and mucosa. EBA includes various distinct clinical manifestations resembling genetic dystrophic epidermolysis bullosa (DEB), Bullous pemphigus, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. These patients have autoantibodies against type VII collagen (C7), an integral component of anchoring fibrils (AFs), which are responsible for attaching the dermis to the epidermis. Destruction or perturbation of the normal functioning AFs clinically results in skin fragility, blisters, erosions, scars, milia, and nail loss, all features reminiscent of genetic dystrophic epidermolysis bullosa. These anti-C7 antibodies are pathogenic because when injected into a mouse, the mouse develops an EBA-like blistering disease. Currently, treatment is often unsatisfactory; however, some success has been achieved with colchicine, dapsone, photopheresis, plasmapheresis, infliximab, rituximab, and IVIG.
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