Evaluation of the Importance of Immunological Profile for Pemphigus Vulgaris in the Light of Necessity to Modify Compensation Theory.

According to the "desmoglein compensation theory," anti-Dsg1 and anti-Dsg3 profiles are crucial for the clinical outcome of pemphigus vulgaris. However, recent studies have highlighted several cases with an incompatibility between the antibody profile and clinical manifestation. Data of 37 patients who had been diagnosed pemphigus vulgaris in our Department between January 2014-June 2016 were retrieved from our clinical database. Patients with ABSIS skin involvement scores, oral mucosa extent and severity scores, anti-Dsg1 and Dsg3 antibody profile were included in this retrospective study. Patients with discordance between clinical manifestations and immunological profile were considered as atypical clinical phenotype. Patients with missing data were excluded. In all 37 patients, Dsg1 and Dsg3 antibody titers at the baseline did not correlate with the concurrent ABSIS scores. At follow up, we detected statistically significant correlations between anti Dsg-1 profile and ABSIS skin involvement scores (p=0.006; r=0.588) and between anti-Dsg3 and ABSIS mucosal extent and severity scores (p=0.058; r=0.431). After treatment, the reduction of Dsg-1 antibody titers was statistically significant in remittent patients (p=0.027). We did not detect statistically significant reduction of Dsg-3 antibodies. Four subjects had incompatible antibody profile and clinical activity. Discordance between phenotype-antibody profile and clinical activity-Dsg titers support the idea that non-Dsg antigens may also be the target for pemphigus autoimmunity.