Bullous Pemphigoid and Neurologic Diseases: Toward a Specific Serologic Profile?

Background: The association of bullous pemphigoid (BP) and neurologic disease (ND) has been proven. Objective: To investigate the presence of specific markers for the association between BP and ND. Method: A total of 47 patients with PB, at the onset of the disease, were retrospectively recruited from January 2015 to October 2017 in a single center (Dermatology Unit, University of Bari “Aldo Moro”, Bari, Italy). Results: We have found an association between BP, ND and specific serologic profile characterized by higher levels of anti-BP180 and anti-BP230 (t(45)=2.319, p=0.025 and t(45)= 2.486, p=0.017, respectively), as compared to BP patients without ND. Furthermore, the univariate analysis revealed a significant association between ND and anti-BP230 positivity (P= 0.043). In detail, we observed a 4-time increased risk to have ND in BP patients showing anti-BP230 positivity. Conclusion: BP230 (BPAG1) is a member of the plakine family that links hemidemosomes to keratin filaments, being expressed at neuronal level. Thus, we hypothesized that alterations induced in ND could lead to the impairment of the “immune privilege”, thus provoking the exposition of BP230 neuronal isoform.