Clinical and Evolutionary Characteristics of Pemphigus by Gender: Comparative Study of 330 Cases

Abstract

Introduction: Pemphigus is a group rare heterogeneous of intraepidermal autoimmune chronic bullous dermatoses associated with a high risk of morbidity and mortality. Despite its rarity, this condition persists as the most prevalent among bullous dermatoses in the Maghreb, including Morocco. Very few studies have investigated the significant influence of gender on the clinical phenotype, the activity score and the evolution of the disease. Our study objective is to analyze   clinical and evolutionary characteristics of pemphigus based on gender differences. Materials and Methods: A comparative retrospective descriptive study was conducted on 142 male and 188 female pemphigus patients treated at the “Department of Dermatology in Ibn Sina University Hospital, Rabat,” between 1990 and 2022. Data from medical records were analyzed, focusing on variables such as age at diagnosis, disease duration, medical history, presence of autoimmune conditions, clinical subtypes, extent of cutaneous and mucosal involvement, and disease progression. Data entry and analysis were performed using Excel and the Statistical Package for the Social Sciences. Results: There were no significant differences in age, and both sexes had the same disease duration prior to diagnosis. The occurrence of pemphigus in women has been more closely associated with autoimmune diseases (25 cases compared to 2 cases in men). Regarding the phenotypes of pemphigus, pemphigus herpetiformis, pemphigus vegetans, and pemphigus vulgaris were more common in women. Isolated mucosal involvement was also more prevalent in women. Women exhibited a more severe Pemphigus Disease Area Index (PDAI) than men. In both male and female patients, the main treatment approach mainly involved oral corticosteroids, followed by an association of corticosteroids and cortisone-sparing agents. Azathioprine was the most frequently administered immunosuppressant (91.6%), with rituximab being used as the initial therapy in 8% of cases. Regarding disease progression, complete remission was noted in 83 women and 52 of men. No notable variances were observed in terms of bleaching time, relapse rates, or mortality. Conclusion: Our findings suggest that gender might impact the clinical presentation of pemphigus patients. Specifically, we observed a higher prevalence of pemphigus herpetiformis, pemphigus vegetans, mucosal involvement, severe Pemphigus Disease Area Index (PDAI), and comorbid autoimmune diseases among women. However, our analysis indicates no significant disparities regarding disease progression or prognosis.