Overview Link to heading
A cross-sectional clinical study examining presentation patterns, diagnostic delays, and treatment outcomes in patients with pemphigus vulgaris, highlighting systemic barriers to early diagnosis.
Project Details Link to heading
Pemphigus vulgaris (PV) is a rare autoimmune blistering disease in which delayed diagnosis can substantially worsen disease severity, recurrence risk, and treatment burden. Despite this, real-world data on diagnostic pathways and delays—particularly in Middle Eastern populations—remain limited.
In this study, I analyzed clinical and epidemiological data from 63 patients with biopsy-confirmed PV treated at tertiary referral centers in Iran. Data were collected via structured telephone interviews and medical record review, focusing on initial symptoms, time to diagnosis, prior consultations, misdiagnoses, treatment patterns, recurrence, and disease control.
Most patients initially presented with mucosal lesions and consulted non-dermatology specialists before receiving a definitive diagnosis. Diagnostic delay was substantial, often exceeding one year, and was significantly associated with higher relapse frequency, greater relapse severity, delayed disease control, and longer time to taper systemic corticosteroids.
The study also characterized real-world treatment patterns, showing widespread use of systemic corticosteroids and rituximab, frequent polypharmacy, and high recurrence rates despite treatment. Earlier diagnosis was consistently associated with fewer relapses and improved clinical trajectories.
This work provides population-specific evidence that diagnostic delay—rather than disease severity alone—is a key driver of poor outcomes in pemphigus vulgaris. The findings support targeted education for non-dermatology clinicians and earlier referral pathways for persistent mucosal lesions. The manuscript has been accepted in Immunity, Inflammation and Disease