Clinical Presentation, Pathogenesis, Diagnosis, and Treatment of Epidermolysis Bullosa Acquisita

Table 3

Mechanisms leading to autoantibody production in EBA.

Mechanism	Demonstrated by	Reference
Genes within MHC locus	(i) HLA haplotype in EBA patients; (ii) MHC association in experimental EBA	[8, 68, 80]
Genes outside MHC locus	(i) Overrepresentation of black-skinned patients in EBA; (ii) Identification of quantitative trait loci (QTL) in experimental EBA	[8, 77]
CD4 T cells	(i) T-cell-deficient nude mice are resistant to induction of immunization-induced EBA; (ii) Time-restricted depletion identifies CD4 T cells to be required for induction of immunization-induced EBA	[66, 83]
HSP90	Blockade of HSP90 prevents induction of and improves already established immunization-induced EBA	[84]