Clinico-demographic characteristics of pemphigus foliaceus: Retrospective analysis of clinic-based data of 103 patients in a non-endemic location.

Background: Pemphigus foliaceus (PF) is an uncommon autoimmune blistering disease characterized by superficial blistering and erosions of the skin. Literature on PF globally is limited. Understanding the clinicodemographic heterogeneity in PF is crucial for offering improved outcomes for affected individuals.

Objective: To study the clinico-demographic characteristics and treatment outcomes of PF patients in north India.

Methods: A retrospective study of patients with PF registered to our clinic between November 2013 and June 2023 at a tertiary care centre in north India was conducted.

Results: A total of 103 patients with PF were included in the study. The mean age at disease onset was 40.4± 15.7 years, with a male-to-female ratio of 1.7:1. The median duration of the disease at presentation was 18 months (ranging from 1 to 240 months). The most commonly prescribed treatment was topical corticosteroids (n=98, 95.1%) or oral corticosteroids (n=97, 94.1%), either alone or in combination with steroid-sparing adjuvants. Rituximab was the commonest adjuvant used in 45 patients (43.6%). Overall, 78 (75.7%) patients achieved clinical remission on minimal therapy (CRmin) in 6.5 ± 3.9 months and clinical remission off therapy (CRoff) was achieved in 10.4 ± 6.0 months in 69 (66.9%) patients. Among patients in the rituximab group, CRmin was achieved in 41 patients (91.1%) in 6.3 ± 4.6 months and CRoff was achieved in 37 cases (82.2%) in 9 ± 5.6 months.

Limitations: The study design is retrospective. Anti-desmoglein antibody titres in the serum were not routinely assessed.

Conclusion: PF in the present study was more common in middle-aged males. Corticosteroids (topical/systemic) formed the mainstay of therapy, with rituximab being the most commonly used steroid-sparing adjuvant. Prospective studies in a larger cohort are required in the future for better understanding of this disease in non-endemic locations.