The demographic attributes, clinical features, and optimal management of 143 patients with pemphigus: A retrospective observational study from a tertiary care center of India

Background: This retrospective study was to understand the clinico-epidemiologic and therapeutic aspects of pemphigus patients attending our clinic. Methods: We analyzed charts of 143 (M: F; 51:92) pemphigus patients having variable severity recorded between 2009 and 2019. Therapies were customized based on patient's age, disease severity, comorbidities, compliance prospects, and affordability. The patients were monitored monthly and as needed for therapeutic outcome in terms of disease control, reduced hospitalization, remission/relapse, and drug toxicity. Results: These patients were aged 15 to 86 years, the majority, 68 (47.5%), was 41 to 60 years of age. The pemphigus vulgaris in 83.9% patients was the commonest variant. Treatment regimens were; dexamethasone-cyclophosphamide-pulse (DCP) therapy in 51.2%, dexamethasone-azathioprine-pulse (DAP) therapy in 11%, dexamethasone-pulse (DP) therapy in 5.5%, rituximab in 24.4%, IVIg in 5.5% patients, and oral corticosteroids with or without adjuvant. Remission occurred after 2-17 (mean 5.8) DCP doses; 14 and 7 patients achieved remission for >= 2 y and >= y, respectively. Rituximab was effective to treat both new and relapsed cases (n = 31). Additional treatment with another adjuvant prolonged remission in seven patients relapsed 12-16 months after treatment with rituximab alone. Overall, oral corticosteroids alone and DAP therapy showed unsatisfactory response. Adverse effects seen in 41.9% of patients were mainly corticosteroids related. Conclusion: The overall clinico-epidemiologic spectrum of pemphigus and therapeutic efficacy of DCP, DAP, or corticosteroids in this study was in sync with the literature. Combining rituximab and corticosteroids plus an immunomodulator initially (phase-1), followed by immunomodulator alone for one year (phase-2) will improve long-term (phase-3) therapeutic outcome. IVIg was effectively useful in patients with concurrent infections.