What is the role of BPAg2 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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Studies from 2006 on autoreactive T and B cells from 35 patients with acute-onset bullous pemphigoid revealed that the percentage of T- cell and B-cell reactivity from these bullous pemphigoid patients against the BPAg2 is much higher than that against BPAg1, further suggesting a more prominent role of BPAg2 in disease development. [20]

Serum levels of autoantibodies against BPAg2 are reportedly correlated with disease activity in some studies. [21] Induction of antibodies against BPAg1 in rabbits does not induce primary blistering, but it can enhance the inflammatory response at the basement membrane. The role of autoantibodies specific for bullous pemphigoid antigens in the initiation and the perpetuation of disease is unknown.

Although BPAg2 has been identified as the major antigen involved with bullous pemphigoid disease development, in 2005, autoantibodies against alpha 6 integrin [22] and laminin-5, [23] 2 other skin basement membrane components, were identified in human patients affected by bullous pemphigoid.

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