Epidermolysis bullosa acquisita: autoimmunity to anchoring fibril collagen.

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.

journal_name

Autoimmunity

journal_title

Autoimmunity

authors

Chen M,Kim GH,Prakash L,Woodley DT

doi

10.3109/08916934.2011.606450

subject

Has Abstract

pub_date

2012-02-01 00:00:00

pages

91-101

issue

1

eissn

0891-6934

issn

1607-842X

journal_volume

45

pub_type

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