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Autoimmunity. 2012 Feb;45(1):91-101. doi: 10.3109/08916934.2011.606450. Epub 2011 Sep 28.

Epidermolysis bullosa acquisita: autoimmunity to anchoring fibril collagen.

Author information

1
Department of Dermatology, The Keck School of Medicine at the University of Southern California, Los Angeles, CA 90033, USA.

Abstract

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.

PMID:
21955050
PMCID:
PMC3411315
DOI:
10.3109/08916934.2011.606450
[Indexed for MEDLINE]
Free PMC Article

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