Therapeutic outcomes of pemphigus in a referral service in Northern Brazil: a retrospective study of 32 patients

Abstract Background Pemphigus are rare chronic autoimmune blistering disorders with challenging therapeutic management. Objective To investigate the therapeutic outcomes of pemphigus patients admitted to a dermatology referral service in northern Brazil. Materials and methods We conducted a retrospective analysis of 32 patients with histopathological diagnosis of pemphigus between 2010 and 2016. Clinical and epidemiological data were collected. Therapeutic outcome was evaluated according to the criteria proposed by the International Pemphigus Committee. Pemphigus Disease Area Index (PDAI) was used to quantify disease severity. Results Of the 32 patients, 68.75% had pemphigus vulgaris (PV) and 31.25% had pemphigus foliaceus (PF). Female-to-male ratio was 1:1. The average age was 49.5 years. All patients received oral prednisone as the first-line therapy. Adjuvant immunosuppressive drugs were gradually added in refractory and/or severe cases. After 24 months of treatment, disease control and complete remission rates were 37.5% and 25%, respectively. The mortality rate was 9.37%. PDAI score was significantly lower at 24 months of therapeutic follow-up (p < .0001). Conclusion Therapeutic management with corticosteroids and/or immunosuppressive drugs was able to induce disease control/complete remission in most of patients. PDAI was a useful tool for objective assessment of disease severity during therapeutic follow-up.