What is the role of autoantibodies in the pathogenesis of bullous pemphigoid (BP)?

Updated: Oct 14, 2020
  • Author: Lawrence S Chan, MD; Chief Editor: Dirk M Elston, MD  more...
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In 2008, a role for IgE class of autoantibodies, particularly those that target BP180, has been established. The higher level of IgE anti-BP180 was correlated with a more severe clinical phenotype. [30]

In an animal model in which C57BL/6 type of mice engrafted with syngeneic mouse skin transgenically expressed human BPAg2 in the epidermal basement membrane zone, antibodies against the human BPAg2 extracellular domain developed first, followed by the occurrence of antibodies to the intracellular domain of the same human BPAg2. Interestingly, the development of later antibodies was associated with the loss of the graft. [31]

IgG autoantibodies from bullous pemphigoid patients are found to deplete cultured keratinocytes of the BPAg2 and weaken cell attachment in vitro, which further supports the pathogenic role of these autoantibodies. [32]

The coagulation cascade is found to be activated in bullous pemphigoid patients, and such activation is found to be correlated with the disease severity and with eosinophilia, suggesting a role of eosinophils in this activation of coagulation, which may contribute to the potential thrombotic risk, as well as inflammation, tissue damage, and blister formation. [33]

A 2010 report of finding anti-BP180 antibodies in unaffected subjects is provides interesting data for further study of the pathogenesis of bullous pemphigoid. [34]

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