Abstract
39 bullous pemphigoid (BP) patients were studied to assess the clinical significance of anti-BP180 and anti-BP230 circulating autoantibodies of BP and correlate their titers with the clinical scores of the BP Disease Area Index (BPDAI) and the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) as well as with the intensity of pruritus measured by the BPDAI pruritus component. All parameters were evaluated by the time of diagnosis (baseline), month 3, and month 6. Titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (r = 0.557, P value < 0.0001) and ABSIS (r = 0.570, P value < 0.0001) values, as well as with BPDAI component for the intensity of pruritus (rho = 0.530, P value = 0.001) at baseline. At month 3, titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (rho = 0.626, P value = 0.000) and ABSIS (rho = 0.625, P value = 0.000) values, as well as with the BPDAI component for the intensity of pruritus (rho = 0.625, P value = 0.000). At month 6, titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (rho = 0.527, P value = 0.001) and ABSIS (rho = 0.526, P value = 0.001) values, as well as with the BPDAI component for the intensity of pruritus (rho = 0.525, P value = 0.001). There was no statistically significant correlation between titers of anti-BP230 autoantibodies and the BPDAI, ABSIS, and BPDAI component for the intensity of pruritus at the same time points.
Highlights
Bullous pemphigoid (BP) is the most common subepidermal autoimmune bullous disease
BP Disease Area Index (BPDAI) has a score of 0–120 for three distinct parameters: number and size of bullous lesions, number and size of erythematous nonbullous lesions, and number and size of mucosal lesions
We aimed to focus on the assessment of disease activity and on the use of the newly introduced clinical scoring systems
Summary
Bullous pemphigoid (BP) is the most common subepidermal autoimmune bullous disease. Detection of anti-BP180 and/or anti-BP230 serum autoantibodies by commercially available ELISA kits has almost become nowadays a routine method for the diagnosis and followup of BP patients [1,2,3].During the last decade, efforts to evaluate the clinical extent and severity of autoimmune bullous diseases, generally, have led to the establishment of scoring systems. Up to today there are two already validated scoring systems for pemphigus, the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and the Pemphigus Disease Area Index (PDAI) [4]. ABSIS has been introduced in 2007, in order to achieve an improved evaluation and monitoring of the status of both oral and cutaneous lesions in patients with pemphigus. It has been used, though, in other autoimmune bullous diseases, such as BP and epidermolysis bullosa acquisita [5]. The Pemphigus Disease Area Index (PDAI) was developed by the International Pemphigus Definitions Committee in 2008, to evaluate both mucosal and cutaneous lesions [6]
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