Clinical and Evolutionary Characteristics of Pemphigus by Gender: Comparative Study of 330 Cases
Published: 2024-03-30
Page: 19-23
Issue: 2024 - Volume 7 [Issue 1]
Amani Fliti *
Department of Dermatology and Venerology, University Hospital Center Ibn Sina, University of Mohamed V, Rabat, Morocco.
Meryem Elomari Alaoui
Department of Dermatology and Venerology, University Hospital Center Ibn Sina, University of Mohamed V, Rabat, Morocco.
Mariame Meziane
Department of Dermatology and Venerology, University Hospital Center Ibn Sina, University of Mohamed V, Rabat, Morocco.
Nadia Ismaili
Department of Dermatology and Venerology, University Hospital Center Ibn Sina, University of Mohamed V, Rabat, Morocco.
Laila Benzekri
Department of Dermatology and Venerology, University Hospital Center Ibn Sina, University of Mohamed V, Rabat, Morocco.
Karima Senouci
Department of Dermatology and Venerology, University Hospital Center Ibn Sina, University of Mohamed V, Rabat, Morocco.
*Author to whom correspondence should be addressed.
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.
Keywords: Pemphigus, gender, bullies dermatosis, autoimmune diseases
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References
Malik AM, Tupchong S, Huang S, Are A, Hsu S, Motaparthi K. An updated review of pemphigus diseases. Medicina (Kaunas). 2021 Oct 9;57(10):1080. DOI: 10.3390/medicina57101080 PMID: 34684117; PMCID: PMC8540565.
Kridin K, Zelber-Sagi S, Bergman R. Pemphigus vulgaris and pemphigus foliaceus: Differences in epidemiology and mortality. Acta Derm. Venereol. 2017; 97:1095-1099. DOI: 10.2340/00015555-2706.
Amagai M, Kárpáti S, Prussick R, Klaus-Kovtun V, Stanley JR. Autoantibodies against the amino-terminal cadherin-like binding domain of pemphigus vulgaris antigen are pathogenic. J. Clin. Investig. 1992;90:919–926. DOI: 10.1172/JCI115968.
Sardana K, Garg V, Agarwal P. Is there an emergent need to modify the desmoglein compensation theory in pemphigus on the basis of Dsg ELISA data and alternative pathogenic mechanisms? Br. J. Dermatol. 2013;168:669–674. DOI: 10.1111/bjd.12012
Timóteo RP, Pessoa-Gonçalves YM, do Carmo Neto JR, Rodrigues WF, da Silva MV, Oliveira CJF. A global view of pemphigus: Geographical variations. Clin Rev Allergy Immunol; 2024 Jan 30. DOI: 10.1007/s12016-024-08980-w Epub ahead of print. PMID: 38289514.
Farah El Hadadi, Mezni Line, Senouci Karima, Laila Benzekri, Nadia Ismaili, Mariame Meziane. Epidemiology of pemphigus: A single center overview in Morocco. International Journal of Dermatology and Venereology; August 31, 2021. DOI: 10.1097/JD9.0000000000000190
Lobo R. Comprehensive gyncology. 5th. Philadelphia, PA: Mosby Elsevier. Menopause: endocrinology, consequences of estrogen deficiency, effects of hormone replacement therapy, treatment regimens. 2007;1039–1071.
Tallab T, Joharji H, Bahamdan K, et al. The incidence of pemphigus in the southern region of Saudi Arabia. Int J Dermatol. 2001;40:570 2.
Kumar KA. Incidence of pemphigus in Thrissur district, south India. Indian J Dermatol Venereol Leprol. 2008;74: 349–51.
Yavuz IH, Yavuz GO, Bayram I, Bilgili SG. Pemphigus in the eastern region of Turkey. Postepy Dermatol Alergol. 2019 Aug;36(4): 455-460. DOI: 10.5114/ada.2019.87449 Epub 2019 Aug 30. PMID: 31616221; PMCID: PMC6791153.
Gazit E, Loewenthal R. The immunogenetics of pemphigus vulgaris. Autoimmun Rev. 2005;4:1620.
Naseer, Sahar Y et al. Gender-based variability in disease presentation in pemphigus vulgaris. Journal of Drugs in Dermatology: JDD. 2014;13(10):1225-30.
Yavuz IH, Yavuz GO, Bayram I, Bilgili SG. Pemphigus in the eastern region of Turkey. Postepy Dermatol Alergol. 2019 Aug;36(4): 455-460.
Zaheri F, Pas HH, Bremer J, et al. Paraneoplastic pemphigus: A detailed case series from the Netherlands revealing atypical cases. J Eur Acad Dermatol Venereol. 2023;37(1):147-153.
DOI:10.1111/jdv.18557
Lagacé F, D'Aguanno K, Prosty C, Laverde-Saad A, Cattelan L, Ouchene L, Oliel S, Genest G, Doiron P, Richer V, Jfri A, O'Brien E, Lefrançois P, Powell M, Moreau L, Litvinov IV, Muntyanu A, Netchiporouk E. The role of sex and gender in dermatology - from pathogenesis to clinical implications. J Cutan Med Surg. 2023 Jul-Aug;27(4):NP1-NP36. DOI: 10.1177/12034754231177582 Epub 2023 Jul 4. PMID: 37401812; PMCID: PMC10486181.