Navigating the Complexities of Pemphigus Vulgaris: A Comprehensive Iranian Study

Area of Science:

• Dermatology
• Autoimmunology
• Epidemiology

Background:

• Pemphigus vulgaris (PV) is a severe autoimmune blistering disease affecting skin and mucous membranes.
• Oral lesions occur in 50%-70% of PV cases, with global incidence varying by region.
• PV typically manifests in the sixth decade, with earlier onset noted in some international populations.

Purpose of the Study:

• To investigate the clinical presentation, diagnosis, and treatment of Pemphigus vulgaris in a specific patient cohort.
• To identify factors contributing to diagnostic delays in Pemphigus vulgaris.

Main Methods:

• A cross-sectional study involving 63 Pemphigus vulgaris patients.
• Data collected via telephone interviews and approved by the Research Ethics Committee.
• Statistical analysis performed using SPSS software.

Main Results:

• The study included 63 patients (56% female), with an average age of 50.17 years and mean age of onset at 44.91 years.
• 70% of patients initially presented with mucosal symptoms, and diagnosis took an average of 17 months.
• Common misdiagnoses included aphthous ulcers and lichen planus; 82% received multiple medications, with prednisolone and rituximab being most frequent.

Conclusions:

• Pemphigus vulgaris frequently begins with mucosal symptoms, often leading to diagnostic delays due to initial consultations with non-dermatologists.
• Diagnostic delays can negatively impact patient outcomes.
• Early recognition of mucosal symptoms is vital for timely Pemphigus vulgaris diagnosis and management.