Autoimmune mucocutaneous blistering diseases in the south of Italy: a 25-year retrospective study on 169 patients.

Abstract

Autoimmune mucocutaneous blistering diseases (AMBDs) represent a heterogeneous group of organ-specific and potentially life-threatening diseases. We sought to determine the relationship between clinical remission and therapeutic regimens with clinical type and phenotype of AMBDs, as well as clinical outcomes achieved based on different therapeutic regimens. A retrospective single-center study on 169 AMBDs patients, including pemphigus vulgaris (PV), mucous membrane pemphigoid (MMP), paraneoplastic autoimmune multiorgan syndrome (PAMS), and lichen planus pemphigoides (LPP), was performed from 1994 to 2019 in an oral medicine tertiary center, where we collected sociodemographic data, clinical type and phenotype, prescribed therapies, and related outcomes. The mean age of AMBDs patients was 55.0±16.4years. They were followed for a mean of 8.4±5.8years. The majority of these patients (62.1%) were successfully managed with conventional immunosuppressive therapy (CIST) alone. However, 37.9% of patients required additional biological treatments, either because they were non-responders or developed severe side effects from CIST, or because of the rapid and severe progression of the disease. Overall, complete clinical remission was achieved in 92.3% of patients. A statistically significant difference was noted between the frequency distribution of AMBDs patients among different therapeutic regimens (P=.002), of different clinical phenotype and type of AMBDs patients and clinical remission (P=.012 and P=.005, respectively). No difference was reported regarding clinical outcomes and different therapeutic regimens. AMBDs' management may be challenging, nonetheless CIST and biologic regimens introduced, when needed as reliable alternatives to CIST, result in a very high percentage of CCR.