Oral Pemphigus Vulgaris Treatment with Corticosteroids and Azathioprine: A Long-Term Study in Shiraz, Iran

BACKGROUND: Treating oral mucosal lesions of Pemphigus Vulgaris (PV) disease is usually challenging for clinicians. We studied the treatment outcomes of the oral PV patients referred to the Oral Medicine Department of Shiraz University of Medical Sciences from 2004 to 2018. METHODS: The medical records of 54 oral PV patients with histopathological confirmation who were treated by a single protocol were studied. The protocol consisted of initial treatment with 1 mg/kg/day of oral prednisolone for all patients. After 4–6 weeks, all patients were prescribed 40 mg of prednisolone. If lesion recovery was not observed or new lesions had developed, adjuvant therapy (maximum dose of 200 mg per day of Azathioprine (AZA)) was initiated anytime during the treatment. The oral prednisolone dosage was gradually tapered to 5 mg/alternate day in 9 months. RESULTS: 47 patients were included in the study. 34.04% were male and 65.96% were female with a mean age of 41.83 ± 12.520. The mean follow-up period was 50.806 ± 44.417 months (over 4 years). The severity of oral involvement was mild in 21.27%, moderate in 36.17%, and severe in 42.6%. During treatment, all patients except one experienced complete remission. The mean time to achieve complete remission was 150.39 ± 224.075 days. Most of the patients experienced relapse due to self-discontinuation of treatment. 55% had complete remission and 43% were in partial remission at the last follow-up session. In 65.96% of patients, treatment-associated side effects were observed. The patients treated with prednisolone alone had significantly more side effects than those using AZA as an adjuvant (80% vs 50%, respectively; P=0.030). The mean duration of follow-ups was longer for patients with side effects (P < 0.01). Topical corticosteroids were used for all patients sometime during the treatment. No deaths were recorded. CONCLUSION: Prescribing low-dose prednisolone and adding AZA in nonresponding cases has good clinical outcomes for the treatment of oral lesions of PV. Adjuvant therapy can avoid the increase in corticosteroid dosage and side effects. The treatment method described in this study can be a helpful guide for clinicians, especially when other immunosuppressive drugs are not available.