Autoimmune Blistering Diseases: Part I. Pemphigus
Authors:
H. Jedličková
Authors‘ workplace:
Dermatovenerologická klinika Fakultní nemocnice u sv. Anny v Brně, přednostka prof. MUDr. Hana Jedličková, Ph. D.
1
Published in:
Čes-slov Derm, 97, 2022, No. 4, p. 144-155
Category:
Reviews (Continuing Medical Education)
Overview
Pemphigus is one of the most serious skin diseases. There are several clinical types that differ in presence of different antibodies against autoantigens, the course and the response to therapy. Knowledge about the pathophysiology of blister formation continues to deepen, and in recent years, the possibilities of therapy have been fundamentally expanded, from classic immunosuppressants to biological therapy with anti-CD20 antibodies. The aim of this article is to briefly present current knowledge about etiopathogenesis, diagnosis, clinic and therapy of this disease.
Keywords:
rituximab – pemphigus – desmoglein
Sources
1. ABRÈU-VELEZ, A. M., HASHIMOTO, T., BOLLAG, W. B. et al. A unique form of endemic pemphigus in northern Colombia. J Am Acad Dermatol, 2003, 49, p. 599–608.
2. AMBER, K. T., VALDEBRAN, M., GRANDO, S. A. Non-Desmoglein Antibodies in Patients With Pemphigus Vulgaris. Frontiers in Immunology, 2018, 9. doi: 10.3389/fimmu.2018.01190.
3. ANDERSEN, K. M., BATES, B. A., RASHIDI, E. A. et al. Long-term use of immunosuppressive medicines and in-hospital COVID-19 outcomes: a retrospective cohort study using data from the National COVID Cohort Collaborative. The Lancet Rheumatology, 2022, 4(1), e33–e41.
4. BOSCH-AMATE, X., IRANZO, P., IVARS, M. et al. Anti- Desmocollin Autoantibodies in Autoimmune Blistering Diseases. Frontiers in Immunology, 2021, 12. doi: 10.3389/fimmu.2021.740820.
5. BYSTRYN, J. C., STEINMAN, N. M. The adjuvant therapy of pemphigus. An update. Arch Dermatol., 1996, 132(2), p. 203–212.
6. COLLIOU, N., PICARD, D., CAILLOT, F. et al. Longterm remissions of severe pemphigus after rituximab therapy are associated with prolonged failure of desmoglein B cell response. Sci Transl Med., 2013, 5, 175ra30.
7. DIDONA, D., MAGLIE, R., EMING, R., HERTL, M. Pemphigus: Current and Future Therapeutic Strategies. Front Immunol., 2019, 10, p. 1418. doi: 10.3389/ fimmu.2019.01418.
8. DOURMISHEV, L. A., GULEVA, D. V., MITEVA, L. G. Intravenous immunoglobulins: mode of action and indications in autoimmune and inflammatory dermatoses. Int J Inflamm., 2016, p. 3523057. doi: 10.1155/2016/3523057.
9. EL-KOMY, M. H., HASSAN, A. S., ABDEL RAHEEM, H. M. et al. Platelet-rich plasma for resistant oral erosions of pemphigus vulgaris: A pilot study. Wound Repair Regen., 2015, 23(6), p. 953–955. doi: 10.1111/ wrr.12363.
10. GHAEDI, F., ETESAMI, I., ARYANIAN, Z. et al. Drug-induced Pemphigus: A Systematic Review of 170 Patients. Int. Immunopharmacology, 2021, 92, 107299. doi: 10.1016/j.intimp.2020.107299.
11. GUALTIERI, B., MARZANO, V., GRANDO, S. A. Atypical pemphigus: autoimmunity against desmocollins and other non-desmoglein autoantigens. Ital J Dermatol Venerol., 2021, 156(2), p.134–141. doi: 10.23736/S2784-8671.20.06619-5.
12. HANS-FILHO, G., AOKI, V., BITTNER, N. R. H., BITTNER, G. C. Fogo selvagem: endemic pemphigus foliaceus. An Bras Dermatol., 2018, 93(5), p. 638–650. doi: 10.1590/abd1806-4841.20188235.
13. HÉBERT, V., BOULARD, C., HOUIVET, E. et al. Large international validation of ABSIS and PDAI pemphigus severity scores. J. Invest. Derm., 2018, 139, p. 31–38. doi: 10.1016/j.jid.2018.04.042.
14. IZUMI, K., BIEBER, K., LUDWIG, R. J. Current Clinical Trials in Pemphigus and Pemphigoid . Front Immunol., 2019, 10. doi: 10.3389/FIMMU.2019.00978.
15. JELTI, L., CORDEL, N., GILLIBERT, A. et al. Incidence and mortality of pemphigus in France. J Invest Dermatol., 2019,139, p. 469–473.
16. JOLY, P., HORVATH, B., PATSATSI, Α. et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol., 2020, 34(9), p. 1900– 1913. doi: 10.1111/jdv.16752.
17. JOLY, P., MAHO-VAILLANT, M., PROST-SQUARCIONI, C. et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet, 2017, 389, p. 2031–2040.
18. KARRAY, M., BADRI, T. Pemphigus Herpetiformis. In: StatPearls [Internet]. Treasure Island (FL): Stat- Pearls Publishing, 2022. Dostupné na www: https:// www.ncbi.nlm.nih.gov/books/NBK482415/.
19. KIM, J. H., KIM, S. C. Paraneoplastic Pemphigus: Paraneoplastic Autoimmune Disease of the Skin and Mucosa. Front Immunol., 2019,10, p. 1259. doi: 10.3389/fimmu.2019.01259.
20. KRIDIN, K., SCHMIDT, E. Epidemiology of Pemphigus. JID Innov., 2021, 20,1(1), p. 100004. doi: 10.1016/j.xjidi.2021.100004.
21. KRIDIN, K., ZELBER-SAGI, S., BERGMAN, R. Pemphigus vulgaris and pemphigus foliaceus: differences in epidemiology and mortality. Acta Derm Venereol., 2017, 97, p. 1095–1099.
22. KRIDIN, K., ZELBER-SAGI, S., COMANESHTER, D. et al. Association between pemphigus and neurologic diseases. JAMA Dermatol., 2018, 154, p. 281–285.
23. KRIDIN, K., SAGI, S. Z., BERGMAN, R. Mortality and Cause of Death in Patients with Pemphigus. Acta Derm Venereol., 2017, 97(5), p. 607–611. doi: 10.2340/00015555-2611.
24. LANGAN, S. M., SMEETH, L., HUBBARD, R. et al. Bullous pemphigoid and pemphigus vulgaris – Incidence and mortality in the UK: population based cohort study. BMJ, 2008, 337, a180.
25. LANDEGREN, N., ISHII, N., ARANDA-GUILLEN, M. et al. A gene-centric approach to biomarker discovery identifies transglutaminase 1 as an epidermal autoantigen. Proc Natl Acad Sci U S A, 2021, 118.
26. LEE, Y. B., LEE, J. H., LEE, S. Y. et al. Incidence and death rate of pemphigus vulgaris and pemphigus foliaceus in Korea: a nationwide, population-based study (2006–2015). J Dermatol., 2018, 45, p. 1396– 1402.
27. LEGER, S., PICARD, D., INGEN-HOUSZ-ORO, S. et al. Prognostic factors of paraneoplastic pemphigus. Arch Dermatol., 2012, 148, p. 1165–1172. doi: 10.1001/archdermatol.2012, 1830.
28. MAO, X., LI, H., SANO, Y. et al. MAPKAP kinase 2 (MK2)-dependent and -independent models of blister formation in pemphigus vulgaris. J Invest Dermatol., 2014, 134, p. 68–76. doi: 10.1038/ jid.2013.224.
29. MEIER, K., HOLSTEIN, J., SOLIMANI, F. et al. Case Report: Apremilast for Therapy-Resistant Pemphigus Vulgaris. Front Immunol., 2020, 11, 588315. doi: 10.3389/fimmu.2020.588315.
30. PAOLINO, G., DIDONA, D., MAGLIULO, G. et al. Paraneoplastic pemphigus: insight into the autoimmune pathogenesis, clinical features and therapy. Int J Mol Sci., 2017, 18, 2532. doi: 10.3390/ ijms18122532.
31. SCHMIDT, E., WASCHKE, J. Apoptosis in pemphigus. Autoimmun Rev., 2009, 8, p. 533–537. doi: 10.1016/j.autrev.2009.01.011.
32. SIGMUND, A. M., WINKLER, M., ENGELMAYER, S. et al. Apremilast prevents blistering in human epidermis by stabilization of keratinocyte adhesion in Pemphigus. Preprint. doi: https://doi. org/10.1101/2022.02.07.478931.
33. SINDHUJA, T., DE, D., HANDA, S. et al. Pemphigus Oral Lesions Intensity Score (POLIS): A Novel Scoring System for Assessment of Severity of Oral Lesions in Pemphigus Vulgaris. Front. Med., 2020. doi. org/10.3389/fmed.2020.00449.
34. SPINDLER, V., EMING, R., SCHMIDT, E. et al. Mechanisms Causing Loss of Keratinocyte Cohesion in Pemphigus. J Invest Dermatol., 2018, 138(1), p. 32– 37. doi: 10.1016/j.jid.2017.06.022.
35. TAKAHASHI, H. Desmoglein 3-reactive B cells “hiding” in pemphigus lesions. J Invest Dermatol., 2017, 137, p. 2255–2257.
36. ULRICHTS, P., GUGLIETTA, A., DREIER, T. et al. Neonatal Fc receptor antagonist efgartigimod safely and sustainably reduces IgGs in humans. J Clin Invest., 2018, 128(10), p. 4372–4386. doi: 10.1172/JCI97911.
37. UZUN, S., DURDU, M., AKMAN, A. et al. Pemphigus in the Mediterranean region of Turkey: a study of 148 cases. Int J Dermatol., 2006, 45, p. 523–528.
38. VODO, D., SARIG, O., SPRECHER, E. The genetics of pemphigus vulgaris. Front Med., 2018, 5, p. 226.
39. WALTER, E., VIELMUTH, F., WANUSKE, M. T. et al. Role of Dsg1- and Dsg3-Mediated Signaling in Pemphigus Autoantibody-Induced Loss of Keratinocyte Cohesion. Front Immunol., 2019, 24,10, 1128. doi: 10.3389/fimmu.2019.01128.
40. WANG, M., LI, F., WANG, X. et al. Features and Risk Factors for Paraneoplastic Autoimmune Multiorgan Syndrome in 145 Chinese Patients. Acta Derm Venereol., 2020, 100: adv00312.
41. WOHL, Y., DREIHER, J., COHEN, A. D. Pemphigus and Osteoporosis: A Case-Control Study. Arch Dermatol., 2010,146(10), p. 1126–1131. doi: 10.1001/archdermatol. 2010.257.
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Dermatology & STDs Paediatric dermatology & STDsArticle was published in
Czech-Slovak Dermatology
2022 Issue 4
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