Clinical Presentation, Diagnosis, and Management of Pemphigus Vulgaris: A Comprehensive Review

Abstract

Background: Pemphigus vulgaris (PV) is a rare, life-threatening autoimmune blistering disorder characterized by the formation of painful blisters and erosions, primarily affecting the skin and mucous membranes. It is caused by the production of autoantibodies against desmogleins, which are key components of the desmosomes that hold epidermal cells together. This study aims to provide a comprehensive overview of the clinical presentation, diagnostic approaches, management strategies, and treatment outcomes in patients with pemphigus vulgaris. Methods: This retrospective, observational study aimed to evaluate the clinical presentation, diagnostic methods, management strategies, and treatment outcomes of pemphigus vulgaris (PV) in 46 patients. The study took place in ODC Healthcare, Green Road, Dhaka, Bangladesh from January 2024 to December 2024. The data were analyzed by SPSS version 25.0. Result: The study included 46 patients, showing a slight male predominance and a median age of 42 years at diagnosis. Oral lesions were the most common clinical feature, observed in 91.3% of patients, followed by skin blisters (82.6%) and erosions (71.7%). Diagnostic methods such as direct immunofluorescence (93.5%) and histopathology (89.1%) were highly effective in confirming the diagnosis. Most patients were treated with corticosteroids (95.7%) and immunosuppressive agents (82.6%), with additional therapies like intravenous immunoglobulin (56.5%) and rituximab (26.1%) used for refractory cases. At follow-up, 26.1% of patients achieved complete remission, 39.1% had partial remission, and 21.7% achieved disease control with medication. However, 13% showed no response to treatment. Conclusion: This study highlights the clinical complexity, diagnostic challenges, and diverse treatment strategies for pemphigus vulgaris (PV). Our findings underscore the predominance of oral lesions and skin blisters in PV presentation, with direct immunofluorescence proving to be the most effective diagnostic tool. Corticosteroids remain the cornerstone of treatment, often combined with immunosuppressive agents and newer therapies like rituximab for refractory cases.