Pemphigoid gestationis: maternal sera recognize epitopes restricted to the N-terminal portion of the extracellular domain of BP180 not present on its shed ectodomain

IgG autoantibodies to type VII collagen and an exclusive IgG3 reactivity to the laminin α3 chain in a patient with an autoimmune subepidermal blistering disease

“…The complement-binding test is a sensitive immunofluorescence assay used to detect complement-fixing autoantibodies to the DEJ in patients with immunobullous diseases. 24 Despite the presence of IgG3 autoantibodies staining the dermal side of the salt-split skin, circulating autoantibodies in the serum of our patient did not exhibit complementfixing properties. Our observation is in line with previous findings showing that the complementactivating capacity of EBA autoantibodies does not correlate with the inflammatory or noninflammatory type of the disease.…”

Induction of dermal-epidermal separation in mice by passive transfer of antibodies specific to type VII collagen

“…Complementfixing activity of antibodies to the DEJ was determined as described (35). Briefly, sections of mouse and human skin were incubated with rabbit IgG and subsequently with fresh mouse serum as a source of complement.…”

Pemphigoid gestationis: Toward a better understanding of the etiopathogenesis

“…14,15 This strong association indicates a pivotal role of MHC class II in the pathogenesis of the disease. 10 25 Another factor contributing to the emergence of PG is possibly an aberrant complement system, with 90% of PG patients carrying the C4 null allele (C4*QO), in contrast to 43% of controls. This may impair the removal of immune complexes and may facilitate their deposition; 26 however, the C4 allele is closely located at the DR alleles on chromosome 6, which consequently leaves a strong disequilibrium, complicating to discern the primary genetic link in PG relevant for disease pathogenesis.…”