What is bullous pemphigoid (BP)?

Updated: Oct 14, 2020
  • Author: Lawrence S Chan, MD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Bullous pemphigoid is a chronic, autoimmune, subepidermal, blistering skin disease that rarely involves mucous membranes. Bullous pemphigoid is characterized by the presence of immunoglobulin G (IgG) autoantibodies specific for the hemidesmosomal bullous pemphigoid antigens BP230 (BPAg1) and BP180 (BPAg2). BP antigen 2 is the usual pathogenic antibody. Occasionally, sublamina densa deposits are noted, related to anti-p200 antibody.

In spontaneous animal models, bullous pemphigoid has been reported to occur in dogs (canine) [9, 10] and horses (equine). [11] Bullous pemphigoid has been found to occur in domestic cats (feline) and Yucatan minipigs (porcine). [12]

In experimental animal models, passive transfer of antibodies to mouse BPAg2 causes blistering in newborn mice similar to that seen in humans. Active induction of anti-BPAg1 antibodies in rabbits enhances inflammation and deposition of immunoreactants at the basement membrane but does not result in spontaneous blistering. [13, 14, 15]

In canine bullous pemphigoid, histologic analysis reveals a subepidermal blistering process with prominent eosinophil infiltration identical to the classic pathology of humans. Similar findings have been observed in feline, [16] porcine, and equine bullous pemphigoid. [11]

As in humans with bullous pemphigoid, the sera from dogs with bullous pemphigoid bind to the epidermal roof of salt-split skin and BP180. The antigenic epitopes of BP180 identified by the canine bullous pemphigoid IgG map to the same epitopes as human bullous pemphigoid autoantibodies. Similar findings were observed in cats, [16] pigs, and horses [11] with bullous pemphigoid.


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