Pemphigus Vulgaris Recurrence Rate and its Relationship with Initial Corticosteroid Dose

Background: Pemphigus vulgaris (PV) is a rare autoimmune blistering disease with a high rate of morbidity and mortality without treatment. Until now, treatments consist mostly of the use of corticosteroid and immunosuppressive drugs. Optimum dosage for corticosteroid therapy is yet to be determined. The aim of the present study was to clear whether different initial corticosteroid therapeutic dosages change the recurrence rate of the disease.Methods: Patients hospitalized with confirmed PV from 2000 to 2006 were enrolled if they received systemic corticosteroids without any adjuvant therapy for no more than three weeks when hospitalized, received their first treatment in the period of the study and were followed for at least two years in the PV clinic of Razi Hospital periodically. Initial steroid dosage as well as the number and type of the recurrence(s) were documented and analyzed.Results: A total of 62 patients with a mean age of 46.8 (± 15.9) were enrolled. Twenty-one patients were female (33.9%) and 41 patients (66.1%) were male. According to the initial dose of systemic corticosteroid, patients were divided into two groups: group A included patients treated with less than 2mg/kg (10 patients or 16.1%) while group B included patients treated with 2mg/kg systemic steroids (52 patients or 83.9%). There was no significant difference in the recurrence rate between the two groups. Recurrence rate was 40% in group A and 51% in group B (P>0.05). According to the type of recurrence, there was a significant difference between the two groups; in group A, the first recurrence was major in 33% of the patients while in group B, this rate was about 28.8% (P<0.05).Conclusion: According to the present study, initial therapeutic dosage did not influence the recurrence rate although the type of recurrence was influenced. Patients treated with higher initial steroid dosages experienced their first episode as a minor recurrence while patients treated with lower initial steroid dosages experienced major recurrences with a higher probability as their first episode.