Navigating the Complexities of Pemphigus Vulgaris: A Comprehensive Iranian Study

Pemphigus vulgaris (PV) is a rare, severe autoimmune disorder characterized by the production of autoantibodies that cause blistering of the skin and mucous membranes, often presenting with oral lesions in 50%-70% of cases. It has a global incidence of 0.5-3.2 per 100,000 people, with variations across regions, and in Iran, the rate is about 1 per 100,000 annually. PV affects both sexes equally and typically manifests in the sixth decade of life, though the age of onset varies internationally, tending to be younger in India and Western countries.

In this cross-sectional study, data were collected from 63 patients diagnosed with PV via telephone interviews. This project was approved by the Research Ethics Committee of Iran University of Medical Sciences. Statistical analyses were performed using SPSS software, version 22.0 (IBM Corp., Armonk, NY, USA).

Among 63 PV patients, 56% were female, and 44% were male, with an average age of 50.17 years and a mean age of onset of 44.91 years (SD = 14.77). Most patients (70%) initially presented with mucosal symptoms, and the average time to diagnosis was approximately 17 months. Common misdiagnoses included aphthous ulcers, lichen planus, and allergic reactions. After diagnosis, most patients (82%) received multiple medications. The most frequently used medications were prednisolone (50 patients, 84.75%), methylprednisolone (10 patients, 16.9%), and rituximab (34 patients, 57.63%).

PV in this cohort most often began with mucosal symptoms and was frequently preceded by consultations with non-dermatology clinicians, contributing to diagnostic delays. Such delays may negatively affect.