Clinical and immunological profile of patients with dipeptidyl peptidase-4 inhibitor-associated bullous pemphigoid

BACKGROUND: Bullous pemphigoid (BP) is an autoimmune blistering disease caused by antibodies against the hemidesmosomal BP180 and/or BP230 proteins. There is an increasing evidence that the use of dipeptidyl peptidase-4 inhibitors, also known as gliptins, increases the risk for BP. The gliptins more frequently associated with BP are vildagliptin and sitagliptin. Clinical, immunological and pathological features of gliptin-associated BP have been reported to be distinct, compared to classic BP.METHODS: In this study, 15 gliptin-associated BP (g-BP) cases have been compared with 16 consecutive idiopathic BP (i-BP) to clarify whether g-BP has distinctive clinical and immunopathological characteristics. Comorbidities, concomitant treatments, latency of the onset of the disease and the time to achieve the remission were also considered.RESULTS: The mean latency from drug intake to g-BP appearance was 9.4 months (median 10, inter-quartile range [IQR] 6-12). There were no differences in sex and age prevalence between the two groups (g-BP median age 77 years, IQR: 70-84; i-BP: 81 years, IQR: 72-86). There were no differences as far clinical presentation including disease severity, lesions types (urticarial and bullous) or mucosal involvement between g-BP and i-BP cases. The median antibody anti-BP180 and anti-BP230 titers was also similar between the two groups with 29.1 UI/mL (IQR: 12.9-65.3) and 11.8 UI/mL (IQR: 1.7-26.3), respectively. Gliptins were withdrawn in ten out of 15 patients and remission was achieved with systemic corticosteroids (0.3-0.7 mg/Kg daily) alone or in association with doxycycline (100-200 mg daily) within a mean of 8 months.CONCLUSIONS: A non-inflammatory phenotype with less erythema, fewer urticarial lesions and fewer eosinophils in skin lesions has been associated with gliptins in selected Japanese BP populations. As reported in European studies, no significant differences among the considered variables in the g-BP and i-BP cases have been found in our study.