A Single-Center Profile of Pemphigus in China: Significant Diagnostic Delay and Evolving Treatment Patterns

Pemphigus encompasses a group of rare and potentially life-threatening autoimmune bullous diseases. Epidemiological research on pemphigus in China remains limited. This study aimed to analyze the clinical characteristics, diagnostic delay, and initial treatment patterns of patients with newly diagnosed pemphigus at a tertiary center in China.

This retrospective study included patients newly diagnosed with pemphigus between January 2020 and December 2024 at the outpatient department of Peking Union Medical College Hospital. Demographic and clinical data were collected and analyzed.

A total of 138 patients were included. Pemphigus vulgaris was the most prevalent subtype (68 cases, 49.3%), followed by pemphigus erythematosus (34 cases, 24.6%), pemphigus foliaceus (13 cases, 9.4%), and pemphigus herpetiformis (11 cases, 8.0%). Notably, the diagnostic delay was substantial, with a median of 5.0 (2.0-12.0) months, and 106 patients (76.8%) had been misdiagnosed before a definitive diagnosis was made. Regarding initial treatment, the most frequently used drugs were corticosteroids (104/132, 78.8%), followed by mycophenolate mofetil (33/132, 25.0%), Tripterygium wilfordii Hook F (29/132, 22.0%), minocycline (28/132, 21.2%), and rituximab (24/132, 18.2%). There was no significant difference among the subtypes in the proportion of patients receiving non-steroidal therapies.

These findings highlight a significant diagnostic delay and outline evolving treatment patterns for pemphigus in a contemporary Chinese cohort. This information may inform future research directions and health policy decisions for managing this rare disease.