Autoimmune Bullous Diseases In Pregnancy: A Systematic Review Of Clinical Presentation, Management, And Outcomes
Keywords:
Clinical Presentation, Autoimmune, Pregnancy, Management, OutcomesAbstract
Background: Autoimmune bullous diseases during pregnancy are rare but clinically significant conditions that require special attention due to pregnancy-related immunological changes and the potential risk of maternal and fetal complications. Objective: This systematic review aimed to summarize the clinical characteristics, management strategies, and maternal–fetal outcomes of autoimmune bullous diseases occurring during pregnancy. Methods: A systematic review was conducted in accordance with the PRISMA 2020 guidelines. Literature searches were performed in PubMed, Scopus, Web of Science, Embase, and the Cochrane Library for studies published between January 2021 and January 2025. From the 309 articles initially identified, 10 studies met the inclusion criteria and were included in the qualitative synthesis. Results: Pemphigoid gestationis was the most frequently reported autoimmune bullous disease, with disease onset predominantly occurring during the second to third trimester of pregnancy. Clinical manifestations varied widely, ranging from mild localized lesions to generalized bullous eruptions. Systemic and topical corticosteroids were the mainstay of treatment and were associated with favorable maternal outcomes in most cases. Reported maternal complications included intrauterine growth restriction (33%), oligohydramnios, and gestational hypertension. Neonatal involvement was observed in approximately 10–17% of cases and was generally self-limiting with a good prognosis. Conclusions: The management of autoimmune bullous diseases during pregnancy requires a multidisciplinary approach to optimize both maternal and fetal outcomes. However, current evidence remains limited, highlighting the need for further studies to establish standardized and evidence-based management protocols.
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Alrifai, N., Puttur, A., Ghanem, F., Dhital, Y., Jabri, A., Al-Abdouh, A., & Alhuneafat, L. (2025). Maternal and fetal outcomes in those with autoimmune connective tissue disease. Clinical Rheumatology, 44(1), 391–401. https://doi.org/10.1007/s10067-024-07242-6
2. Atalay, N. E., Dağıstanlı, F., Şimşek, H. U., & Caliskan, E. (2025). Evaluation Of Maternal And Fetal Findings In Patients With Pemphigoid Gestationis. International Journal of Gynaecology and Obstetrics: The Official Organ of the International Federation of Gynaecology and Obstetrics, 169(1), 274–278. https://doi.org/10.1002/ijgo.16086
3. Baban, F., Xie, F., Lehman, Julia S., Theiler, R., Todd, A., Davis, Dawn M., & Johnson, Emma F.. (2023). Histopathological features of pemphigoid gestationis and polymorphic eruption of pregnancy: A blinded retrospective comparative study of 31 cases. Journal of Cutaneous Pathology, 50(4), 358–363. https://doi.org/10.1111/cup.14358
4. Bramer, W. M., Rethlefsen, M. L., Kleijnen, J., & Franco, O. H. (2017). Optimal database combinations for literature searches in systematic reviews: A prospective exploratory study. Systematic Reviews, 6(1), 1–12. https://doi.org/10.1186/s13643-017-0644-y
5. Deeks, J. J., Bossuyt, P. M., Leeflang, M. M., & Takwoingi, Y. (2023). Cochrane Handbook for Systematic Reviews of Diagnostic Test Accuracy. Wiley. https://books.google.co.id/books?id=_C_FEAAAQBAJ
6. Fagundes, P. P. S., Santi, C. G., Maruta, C. W., Miyamoto, D., & Aoki, V. (2021). Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach. Anais Brasileiros de Dermatologia, 96(5), 581–590. https://doi.org/10.1016/j.abd.2020.10.007
7. Feng, X., Zheng, H., Wang, M., Wang, Y., Zhou, X., Zhang, X., Li, J., Xiao, Y., Wei, M., Li, X., Hashimoto, T., Li, J., & Li, W. (2025). Autoimmune bullous diseases: pathogenesis and clinical management. Molecular Biomedicine, 6(1). https://doi.org/10.1186/s43556-025-00272-9
8. Genovese, G., Derlino, F., Berti, E., & Marzano, A. V. (2020). Treatment of Autoimmune Bullous Diseases During Pregnancy and Lactation: A Review Focusing on Pemphigus and Pemphigoid Gestationis. Frontiers in Pharmacology, 11(October), 1–8. https://doi.org/10.3389/fphar.2020.583354
9. Higgins, J. P. T., Thompson, S. G., Deeks, J. J., & Altman, D. G. (2003). Measuring inconsistency in meta-analyses. BMJ (Clinical Research Ed.), 327(7414), 557–560. https://doi.org/10.1136/bmj.327.7414.557
10. Inatomi, A., Katsura, D., Tokoro, S., Tsuji, S., & Murakami, T. (2024). Perinatal Outcome of Pemphigoid Gestationis: A Report of Three Cases and Review of the Literature. Cureus, 16(9), 1–8. https://doi.org/10.7759/cureus.68582
11. Jiao, H. N., Ruan, Y. P., Liu, Y., Pan, M., & Zhong, H. P. (2021). Diagnosis, fetal risk and treatment of pemphigoid gestationis in pregnancy: A case report. World Journal of Clinical Cases, 9(34), 10645–10651. https://doi.org/10.12998/wjcc.v9.i34.10645
12. Kajdas, A. A., Żebrowska, A., Zalewska-Janowska, A., & Czerwonogrodzka-Senczyna, A. (2024). The Role of Nutrition in the Pathogenesis and Treatment of Autoimmune Bullous Diseases—A Narrative Review. Nutrients , 16(22), 1–23. https://doi.org/10.3390/nu16223961
13. Kasperkiewicz, M., & Woodley, D. T. (2020). COVID-19 and autoimmune bullous diseases: Lessons learned. Autoimmunity Reviews, January. https://doi.org/10.1016/j.autrev.2023.103286 Received
14. Ma, L.-L., Wang, Y.-Y., Yang, Z.-H., Huang, D., Weng, H., & Zeng, X.-T. (2020). Methodological quality (risk of bias) assessment tools for primary and secondary medical studies: what are they and which is better? Military Medical Research, 7(7), 1–11. https://mmrjournal.biomedcentral.com/track/pdf/10.1186/s40779-020-00238-8.pdf
15. Munn, Z., Stone, J. C., Aromataris, E., Klugar, M., Sears, K., Leonardi-Bee, J., & Barker, T. H. (2022). Assessing the risk of bias of quantitative analytical studies: introducing the vision for critical appraisal within JBI systematic reviews. JBI Evidence Synthesis, 21(3), 467–471. https://doi.org/10.11124/JBIES-22-00224
16. Nekrasova, E., Muraveva, E., Dubensky, V., Aleksandrova, O., Bogdanova, N., & Sargsyan, A. (2025). Bullous pemphigoid in pregnant women: Clinical observation. Euromedica Archive, 15(2). https://doi.org/10.35630/2025/15/2.213
17. Paez, A. (2017). Gray literature: An important resource in systematic reviews. Journal of Evidence-Based Medicine, 10(3), 233–240. https://doi.org/10.1111/jebm.12266
18. Page, M. J., McKenzie, J. E., Bossuyt, P. M., Boutron, I., Hoffmann, T. C., Mulrow, C. D., Shamseer, L., Tetzlaff, J. M., Akl, E. A., Brennan, S. E., Chou, R., Glanville, J., Grimshaw, J. M., Hróbjartsson, A., Lalu, M. M., Li, T., Loder, E. W., Mayo-Wilson, E., McDonald, S., ... Moher, D. (2021). The PRISMA 2020 statement: An updated guideline for reporting systematic reviews. Bmj, 372. https://doi.org/10.1136/bmj.n71
19. Parfene, C., Bohiltea, R., Mihai, B., Grigoriu, C., Margaritescu, I., Chirita, A., Zugravu, C., & Pelinescu-Onciul, D. (2021). Influence of pemphigoid gestationis on pregnancy outcome: A case report and review of the literature. Experimental and Therapeutic Medicine, 23(1), 1–5. https://doi.org/10.3892/etm.2021.10945
20. Patel, S., & Okon, L. (2025). A Rare Case of Pemphigoid Gestationis. SKIN: Journal of Cutaneous Medicine, 9(1), 2139–2144. https://doi.org/10.25251/SKIN.9.1.18
21. Popay, J., Roberts, H., & Sowden, A. (2006). Guidance on the conduct of narrative synthesis in systematic reviews. Accessed 22nd May, April 2006, 1–92.
22. Rethlefsen, M. L., Kirtley, S., Waffenschmidt, S., Page, M. J., & Koffe, J. B. (2021). PRISMA-S: an extension to the PRISMA Statement for Reporting Literature Searches in Systematic Reviews. Systematic Reviews, 30, 385–386. https://doi.org/10.1007/978-3-642-71213-5_65
23. Tigges, M., Dräger, S., Piccini, I., Bieber, K., Vorobyev, A., Edelkamp, J., Bertolini, M., & Ludwig, R. J. (2025). Pemphigoid disease model systems for clinical translation. Frontiers in Immunology, 16(March), 1–25. https://doi.org/10.3389/fimmu.2025.1537428
24. Vičić, M., & Marinović, B. (2023). Autoimmune bullous diseases in pregnancy: an overview of pathogenesis, clinical presentations, diagnostics and available therapies. Italian Journal of Dermatology and Venereology, 158(2), 99–109. https://doi.org/10.23736/S2784-8671.23.07553-9
25. Waffenschmidt, S., Knelangen, M., Sieben, W., Bühn, S., & Pieper, D. (2019). Single screening versus conventional double screening for study selection in systematic reviews: A methodological systematic review. BMC Medical Research Methodology, 19(1), 1–9. https://doi.org/10.1186/s12874-019-0782-0
26. Xie, F., Davis, D. M. R., Baban, F., Johnson, E. F., Theiler, R. N., Todd, A., Pruneddu, S., Murase, J. E., Maul, J.-T., Ambros-Rudolph, C. M., & Lehman, J. S. (2023). Development and multicenter international validation of a diagnostic tool to differentiate between pemphigoid gestationis and polymorphic eruption of pregnancy. Journal of the American Academy of Dermatology, 89(1), 106–113. https://doi.org/10.1016/j.jaad.2023.01.027
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