Dapsone and Sulfasalazine as Adjuvant Therapies in Pemphigus Vulgaris: A Systematic Review

Background: Adjuvant treatments are often employed in pemphigus vulgaris (PV) to reduce dependence on corticosteroids. When first-line adjuvants are ineffective, considering alternative adjuvant therapies may be necessary. Dapsone and sulfasalazine represent two options. Objective: To evaluate the use of dapsone and sulfasalazine in the treatment of PV. Methods: A PubMed search identified patients with PV treated with dapsone or sulfasalazine. We included all studies with ≥3 patients published in English between 1980 and December 2022. Clinical improvement was defined as disease control or complete remission (when adjuvant therapy was initiated in uncontrolled disease) and as a reduction of corticosteroids to ≤7.5 mg per day (when adjuvant therapy was initiated in controlled disease). Results: Eleven studies were identified representing 75 patients treated with dapsone and 66 patients treated with sulfasalazine. Six patients received dapsone monotherapy, and all had clinical improvement. Sixty-nine received adjuvant dapsone, leading to clinical improvement in 40 (58%). Fifty-seven patients received sulfasalazine as an adjuvant to intravenous corticosteroids, leading to clinical improvement in 49 (86%). The remaining 9 patients received sulfasalazine following rituximab, and all had clinical improvement. Conclusions: Dapsone and sulfasalazine have evidence supporting their efficacy and can be considered when first-line adjuvant therapies are unsuccessful.