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Epidermolysis Bullosa Acquisita
Synonyms
Acquired epidermolysis bullosa; Dermolytic pemphigoid; EBA
Definition and Characteristics
An autoimmune subepidermal blistering disease, associated with autoreactivity against collagen VII, a component of anchoring fibrils. The classical mechanobullous presentation of epidermolysis bullosa acquisita (EBA) consists of skin fragility, noninflammatory blistering and healing with scars or milia, but inflammatory disseminated forms resembling the pemphigoid diseases have been described [1].
Prevalence
In Western Europe the annual incidence has been estimated to be 0.25 per million. The prevalence is unclear.
Genes
Susceptibility to develop EBA appears to be increased in Caucasians, Africans and Americans with HLA class II alleles DRB1*1501 and DR5.
Molecular and Systemic Pathophysiology
Collagen VII represents the major component of anchoring fibrils located at the dermoepidermal junction (see Fig. 1, in Bullous pemphigoid). The N-terminal