Introduction: Pemphigus vulgaris is a rare, autoimmune disorder characterized by blistering skin and mucous membrane lesions, 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. The disease typically manifests in the 6th decade of life, though the age of onset varies internationally, being younger in India and Western countries. Method: In this cross-sectional study, information of 63 patients, diagnosed with pemphigus vulgaris was confirmed by biopsy of the lesions, was collected during a telephone call. This project was approved by the Research Ethics Committee of Iran University of Medical Sciences. SPSS version 22 software was used for the statistical analysis of the data. Results: In a study of 63 pemphigus vulgaris patients, 56% were female and 44% male, with an average age of 50.17 years. Most patients initially presented with mucosal symptoms (70%), and the average time to diagnosis was about 17 months. Common misdiagnoses included aphthous ulcers, lichen planus, and allergic reactions. Post-diagnosis, most patients (82%) received multiple medications. Specific treatments included prednisolone (12%), methylprednisolone (1.7%), and methotrexate (1.7%). Also Those who initially managed their illness well tended to have more frequent and intense flare-ups later. Patients diagnosed early generally had fewer relapses, while those who first showed mucosal symptoms experienced longer periods without relapses. Also, being diagnosed late can affect how well treatments work and how the disease is managed. Discussion: Pemphigus vulgaris predominantly affects middle-aged women, typically emerging in their fifth decade, with mucosal symptoms often preceding skin manifestations. Delayed diagnosis, particularly in cases presenting with skin symptoms, may impact prognosis. Treatment choice and adjuvant therapy post-diagnosis may influence relapse rates, warranting further investigation.

Background: Autoimmune bullous diseases (AIBDs) are a group of rare blistering dermatoses of the mucous membrane and/or skin. The efficacy, safety, and treatment durability of intravenous immunoglobulin (IVIg) as an alternative treatment should be explored. Objectives: To systematically review the available literature regarding treatment outcomes with IVIg in AIBD patients. Methods: The predefined search strategy was incorporated into the following database, MEDLINE/PubMed, Embase, Scopus, and Web of Science on 18th July 2022. Sixty studies were enrolled using Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Results: The use of IVIg alone or combined with rituximab was reported in 500 patients with pemphigus, 82 patients with bullous pemphigoid, 146 patients with mucous membranes pemphigoid, and 19 patients with epidermolysis bullosa acquisita. IVIg therapy induced disease remission of 82.8% in pemphigus, 88% in bullous pemphigoid, 91.3% in mucous membranes pemphigoid, and 78.6% in epidermolysis bullosa acquisita. The combination therapy of RTX+IVIg led to disease remission of 86.7% in pemphigus, 100% in bullous pemphigoid, and 75.0% in mucous membranes pemphigoid. Among all the included patients, 37.5% experienced at least one IVIg-related side effect; the most common ones were headaches, fever/chills, and nausea/vomiting. Conclusions: The use of IVIg with or without rituximab had a favorable clinical response in patients with AIBDs. IVIg has no major influence on the normal immune system, which makes its utilization for patients with AIBDs reasonable.