Bullous pemphigoid (BP) is an autoimmune bullous disease characterized by autoantibody production against BP180 and BP230. Two scoring systems have been validated for BP including: Bullous Pemphigoid Disease Area Index (BPDAI) and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). In this study, we investigated correlations between both scoring systems and either anti-BP180 NC16A or anti-BP230 values. BPDAI and ABSIS were used to measure disease activity in 95 BP patients at Razi Hospital in Tehran, Iran. ELISA was performed on patient sera to identify any significant relationship between anti-BP180 NC16A/anti-BP230 values and BP disease activity. The two scores showed a strong correlation (ρ = 0.73; p value < 0.0001). Anti-BP180 NC16A values correlated with BPDAI (ρ = 0.49, p value = 0.0001), ABSIS (ρ = 0.47, p value < 0.0001), and BPDAI-Pruritus scores (ρ = 0.29, p value < 0.005). There was a strong correlation between anti-BP180 NC16A values and the ABSIS Skin score (ρ = 0.58, p value < 0.0001), and a moderate correlation with erosion/blister BPDAI score (ρ = 0.48, p value < 0.001) and urticaria/erythema BPDAI score (ρ = 0.27, p value = 0.009). Anti-BP230 values did not demonstrate any relationship with either scores or their subcomponents. Both scoring systems demonstrated moderate validity. Mucosal components did not show any correlation with anti-BP antibodies and are disproportionately presented in both BP scoring tools.
Keywords: Anti-BP180 NC16A; Anti-BP230; Autoimmune Bullous Skin Disorder Intensity Score; Bullous Pemphigoid Disease Area Index; Bullous pemphigoid.