Autoantibodies to a 200-kDa protein in the lamina lucida are responsible for the disease. Studies have tried to characterize this protein, but the protein remains unidentified at this time. Suspicion exists for involvement of anti-laminin γ1 antibodies in disease pathogenesis. However, no correlation has been found between anti-laminin γ1 antibodies and blister formation in ex vivo studies.
Some believe anti-p200 pemphigoid may follow a milder course than other autoimmune bullous disorders. However, more recently, a spectrum of disease severity has been recognized, with treatment-resistant cases having also been reported.
L12.0 – Bullous pemphigoid
77090002 – Bullous pemphigoid
Consider several subepidermal blistering diseases in the differential:
- BP, especially the dyshidrosiform variant
- EBA, especially the inflammatory variant
- Mucous membrane pemphigoid (MMP)
- Bullous systemic lupus erythematosus
- Dermatitis herpetiformis
- Linear IgA dermatosis