Spandidos Publications Logo
  • About
    • About Spandidos
    • Aims and Scopes
    • Abstracting and Indexing
    • Editorial Policies
    • Reprints and Permissions
    • Job Opportunities
    • Terms and Conditions
    • Contact
  • Journals
    • All Journals
    • Oncology Letters
      • Oncology Letters
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • International Journal of Oncology
      • International Journal of Oncology
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • Molecular and Clinical Oncology
      • Molecular and Clinical Oncology
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • Experimental and Therapeutic Medicine
      • Experimental and Therapeutic Medicine
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • International Journal of Molecular Medicine
      • International Journal of Molecular Medicine
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • Biomedical Reports
      • Biomedical Reports
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • Oncology Reports
      • Oncology Reports
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • Molecular Medicine Reports
      • Molecular Medicine Reports
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • World Academy of Sciences Journal
      • World Academy of Sciences Journal
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • International Journal of Functional Nutrition
      • International Journal of Functional Nutrition
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • International Journal of Epigenetics
      • International Journal of Epigenetics
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • Medicine International
      • Medicine International
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
  • Articles
  • Information
    • Information for Authors
    • Information for Reviewers
    • Information for Librarians
    • Information for Advertisers
    • Conferences
  • Language Editing
Spandidos Publications Logo
  • About
    • About Spandidos
    • Aims and Scopes
    • Abstracting and Indexing
    • Editorial Policies
    • Reprints and Permissions
    • Job Opportunities
    • Terms and Conditions
    • Contact
  • Journals
    • All Journals
    • Biomedical Reports
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • Experimental and Therapeutic Medicine
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • International Journal of Epigenetics
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • International Journal of Functional Nutrition
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • International Journal of Molecular Medicine
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • International Journal of Oncology
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • Medicine International
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • Molecular and Clinical Oncology
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • Molecular Medicine Reports
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • Oncology Letters
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • Oncology Reports
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
    • World Academy of Sciences Journal
      • Information for Authors
      • Editorial Policies
      • Editorial Board
      • Aims and Scope
      • Abstracting and Indexing
      • Bibliographic Information
      • Archive
  • Articles
  • Information
    • For Authors
    • For Reviewers
    • For Librarians
    • For Advertisers
    • Conferences
  • Language Editing
Login Register Submit
  • This site uses cookies
  • You can change your cookie settings at any time by following the instructions in our Cookie Policy. To find out more, you may read our Privacy Policy.

    I agree
Search articles by DOI, keyword, author or affiliation
Search
Advanced Search
presentation
Experimental and Therapeutic Medicine
Join Editorial Board Propose a Special Issue
Print ISSN: 1792-0981 Online ISSN: 1792-1015
Journal Cover
November-2021 Volume 22 Issue 5

Full Size Image

Sign up for eToc alerts
Recommend to Library

Journals

International Journal of Molecular Medicine

International Journal of Molecular Medicine

International Journal of Molecular Medicine is an international journal devoted to molecular mechanisms of human disease.

International Journal of Oncology

International Journal of Oncology

International Journal of Oncology is an international journal devoted to oncology research and cancer treatment.

Molecular Medicine Reports

Molecular Medicine Reports

Covers molecular medicine topics such as pharmacology, pathology, genetics, neuroscience, infectious diseases, molecular cardiology, and molecular surgery.

Oncology Reports

Oncology Reports

Oncology Reports is an international journal devoted to fundamental and applied research in Oncology.

Experimental and Therapeutic Medicine

Experimental and Therapeutic Medicine

Experimental and Therapeutic Medicine is an international journal devoted to laboratory and clinical medicine.

Oncology Letters

Oncology Letters

Oncology Letters is an international journal devoted to Experimental and Clinical Oncology.

Biomedical Reports

Biomedical Reports

Explores a wide range of biological and medical fields, including pharmacology, genetics, microbiology, neuroscience, and molecular cardiology.

Molecular and Clinical Oncology

Molecular and Clinical Oncology

International journal addressing all aspects of oncology research, from tumorigenesis and oncogenes to chemotherapy and metastasis.

World Academy of Sciences Journal

World Academy of Sciences Journal

Multidisciplinary open-access journal spanning biochemistry, genetics, neuroscience, environmental health, and synthetic biology.

International Journal of Functional Nutrition

International Journal of Functional Nutrition

Open-access journal combining biochemistry, pharmacology, immunology, and genetics to advance health through functional nutrition.

International Journal of Epigenetics

International Journal of Epigenetics

Publishes open-access research on using epigenetics to advance understanding and treatment of human disease.

Medicine International

Medicine International

An International Open Access Journal Devoted to General Medicine.

Journal Cover
November-2021 Volume 22 Issue 5

Full Size Image

Sign up for eToc alerts
Recommend to Library

  • Article
  • Citations
    • Cite This Article
    • Download Citation
    • Create Citation Alert
    • Remove Citation Alert
    • Cited By
  • Similar Articles
    • Related Articles (in Spandidos Publications)
    • Similar Articles (Google Scholar)
    • Similar Articles (PubMed)
  • Download PDF
  • Download XML
  • View XML
Review Open Access

Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review)

  • Authors:
    • Victor-Vlad Costan
    • Cristina Popa
    • Mădălina Florina Hâncu
    • Elena Porumb‑Andrese
    • Mihaela Paula Toader
  • View Affiliations / Copyright

    Affiliations: Department of Oral and Maxillofacial Surgery, ‘Grigore T. Popa’ University of Medicine and Pharmacy, 700115 Iași, Romania, Department of Oral Medicine and Oral Dermatology, ‘Grigore T. Popa’ University of Medicine and Pharmacy, 700115 Iași, Romania, Department of Dermatology, ‘Sf. Spiridon’ Clinical Emergency County Hospital, 700111 Iași, Romania, Department of Dermatology, ‘Grigore T. Popa’ University of Medicine and Pharmacy, 700115 Iași, Romania
    Copyright: © Costan et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
  • Article Number: 1335
    |
    Published online on: September 20, 2021
       https://doi.org/10.3892/etm.2021.10770
  • Expand metrics +
Metrics: Total Views: 0 (Spandidos Publications: | PMC Statistics: )
Metrics: Total PDF Downloads: 0 (Spandidos Publications: | PMC Statistics: )
Cited By (CrossRef): 0 citations Loading Articles...

This article is mentioned in:



Abstract

Pemphigus represents a group of chronic inflammatory disorders characterized by autoantibodies that target components of desmosomes, leading to the loss of intercellular adhesion between keratinocytes and causing intraepithelial blistering. The pemphigus group consists of four main clinical types with several variants: pemphigus vulgaris (with pemphigus vegetans and pemphigus herpetiformis as variants), pemphigus foliaceus, paraneoplastic pemphigus and IgA pemphigus (with two clinical variants: intraepidermal neutrophilic IgA dermatosis and subcorneal pustular dermatosis). Genetic factors are involved in the pathogenesis, with HLA‑DR4 (DRB1*0402) and HLA‑DRw6 (DQB1*0503) allele more common in patients with pemphigus vulgaris, HLA class II DRB1*0344 and HLA Cw*1445 correlated with paraneoplastic pemphigus, and HLA‑DRB1*04:01, HLA‑DRB1*04:06, HLA‑DRB1*01:01, HLA‑DRB1*14, associated with a higher risk of developing pemphigus foliaceus. Autoantibodies are conducted against structural desmosomal proteins in the skin and mucous membranes, mainly desmogleins, desmocollins and plakins. Cell‑mediated immunity may also play a role, especially in paraneoplastic pemphigus. Patients may present erythema, blisters, erosions, and ulcers that may affect the skin, as well as mucosal surfaces of the oral cavity, eyes, nose, leading to severe complaints including pain, dysphagia, and fetor. Oral mucosal postbullous erosive lesions are frequently the first sign of disease in pemphigus vulgaris and in paraneoplastic pemphigus, without skin involvement, making the diagnosis difficult. Treatment options classically include immunosuppressive agents, such as corticosteroids and corticosteroid‑sparing agents such as azathioprine, mycophenolate mofetil, cyclophosphamide, methotrexate or dapsone. Newer therapies focus on blocking cell signaling events induced by pathogenic autoantibodies and/or targeting specific autoantibodies. The disease evolution is conditioned by the treatment with maximum doses of corticosteroids and the side effects associated with long‑term immunosuppressive therapy, which is why patients need a multidisciplinary approach in following the treatment. In this review, we provide a comprehensive overview of the epidemiology, pathophysiology, clinical aspect, diagnosis and management of the main intraepidermal blistering diseases from the pemphigus group.
View Figures

Figure 1

Figure 2

Figure 3

View References

1 

Lakoš Jukić I, Jerković Gulin S and Marinović B: Blistering diseases in the mature patient. Clin Dermatol. 36:231–238. 2018.PubMed/NCBI View Article : Google Scholar

2 

Kasperkiewicz M: COVID-19 outbreak and autoimmune bullous diseases: A systematic review of published cases. J Am Acad Dermatol. 84:563–568. 2021.PubMed/NCBI View Article : Google Scholar

3 

Chaudhari P and Marinkovich MP: What's new in blistering disorders? Curr Allergy Asthma Rep. 7:255–263. 2007.PubMed/NCBI View Article : Google Scholar

4 

Hofmann SC, Juratli HA and Eming R: Bullous autoimmune dermatoses. J Dtsch Dermatol Ges. 16:1339–1358. 2018.PubMed/NCBI View Article : Google Scholar

5 

Bickle K, Roark TR and Hsu S: Autoimmune bullous dermatoses: A review. Am Fam Physician. 65:1861–1870. 2002.PubMed/NCBI

6 

Patrício P, Ferreira C, Gomes MM and Filipe P: Autoimmune bullous dermatoses: A review. Ann NY Acad Sci. 1173:203–210. 2009.PubMed/NCBI View Article : Google Scholar

7 

Egami S, Yamagami J and Amagai M: Autoimmune bullous skin diseases, pemphigus and pemphigoid. J Allergy Clin Immunol. 145:1031–1047. 2020.PubMed/NCBI View Article : Google Scholar

8 

Kneisel A and Hertl M: Autoimmune bullous skin diseases. Part 1: Clinical manifestations. J Dtsch Dermatol Ges. 9:844–856; quiz 857. 2011.PubMed/NCBI View Article : Google Scholar : (In English, German).

9 

Kasperkiewicz M, Ellebrecht CT, Takahashi H, Yamagami J, Zillikens D, Payne AS and Amagai M: Pemphigus. Nat Rev Dis Primers. 3(17026)2017.PubMed/NCBI View Article : Google Scholar

10 

Pollmann R, Schmidt T, Eming R and Hertl M: Pemphigus: A comprehensive review on pathogenesis, clinical presentation and novel therapeutic approaches. Clin Rev Allergy Immunol. 54:1–25. 2018.PubMed/NCBI View Article : Google Scholar

11 

Melchionda V and Harman KE: Pemphigus vulgaris and pemphigus foliaceus: An overview of the clinical presentation, investigations and management. Clin Exp Dermatol. 44:740–746. 2019.PubMed/NCBI View Article : Google Scholar

12 

Didona D, Maglie R, Eming R and Hertl M: Pemphigus: Current and future therapeutic strategies. Front Immunol. 10(1418)2019.PubMed/NCBI View Article : Google Scholar

13 

Oktarina DA, van der Wier G, Diercks GF, Jonkman MF and Pas HH: IgG-induced clustering of desmogleins 1 and 3 in skin of patients with pemphigus fits with the desmoglein nonassembly depletion hypothesis. Br J Dermatol. 165:552–562. 2011.PubMed/NCBI View Article : Google Scholar

14 

Amber KT, Valdebran M and Grando SA: Non-desmoglein antibodies in patients with pemphigus vulgaris. Front Immunol. 9(1190)2018.PubMed/NCBI View Article : Google Scholar

15 

Yan L, Wang JM and Zeng K: Association between HLA-DRB1 polymorphisms and pemphigus vulgaris: A meta-analysis. Br J Dermatol. 167:768–777. 2012.PubMed/NCBI View Article : Google Scholar

16 

Sultan AS, Villa A, Saavedra AP, Treister NS and Woo SB: Oral mucous membrane pemphigoid and pemphigus vulgaris-a retrospective two-center cohort study. Oral Dis. 23:498–504. 2017.PubMed/NCBI View Article : Google Scholar

17 

Mustafa MB, Porter SR, Smoller BR and Sitaru C: Oral mucosal manifestations of autoimmune skin diseases. Autoimmun Rev. 14:930–951. 2015.PubMed/NCBI View Article : Google Scholar

18 

Rashid H, Lamberts A, Diercks GFH, Pas HH, Meijer JM, Bolling MC and Horváth B: Oral lesions in autoimmune bullous diseases: An Overview of clinical characteristics and diagnostic algorithm. Am J Clin Dermatol. 20:847–861. 2019.PubMed/NCBI View Article : Google Scholar

19 

Carvalho AA, Santos Neto DAD, Carvalho MADR, Eleutério SJP and Xavier AREO: Neonatal pemphigus in an infant born to a mother with pemphigus vulgaris: A case report. Rev Paul Pediatr. 37:130–134. 2019.PubMed/NCBI View Article : Google Scholar

20 

Panko J, Florell SR, Hadley J, Zone J, Leiferman K and Vanderhooft S: Neonatal pemphigus in an infant born to a mother with serologic evidence of both pemphigus vulgaris and gestational pemphigoid. J Am Acad Dermatol. 60:1057–1062. 2009.PubMed/NCBI View Article : Google Scholar

21 

Amer YB and Al Ajroush W: Pemphigus vulgaris in a neonate. Ann Saudi Med. 27:453–455. 2007.PubMed/NCBI View Article : Google Scholar

22 

Huang YH, Wang SH, Kuo TT and Chi CC: Pemphigus vegetans occurring in a split-thickness skin graft. Dermatol Surg. 31:240–243. 2005.PubMed/NCBI View Article : Google Scholar

23 

Sillevis Smitt JH, Mulder TJ, Albeda FW and Van Nierop JC: Pemphigus vegetans in a child. Br J Dermatol. 127:289–291. 1992.PubMed/NCBI View Article : Google Scholar

24 

Jain V, Jindal N and Imchen S: Localized pemphigus vegetans without mucosal involvement. Indian J Dermatol. 59(210)2014.PubMed/NCBI View Article : Google Scholar

25 

Son YM, Kang HK, Yun JH, Roh JY and Lee JR: The Neumann type of pemphigus vegetans treated with combination of dapsone and steroid. Ann Dermatol. 23 (Suppl 3):S310–S313. 2011.PubMed/NCBI View Article : Google Scholar

26 

Peterman CM, Vadeboncoeur S, Schmidt BA and Gellis SE: Pediatric pemphigus herpetiformis: Case report and review of the literature. Pediatr Dermatol. 34:342–346. 2017.PubMed/NCBI View Article : Google Scholar

27 

Shimizu Y, Wakabayashi K, Hayashi Y, Hara K, Aoyama R, Niimi T, Tomino Y, Wada R, Hata M and Suzuki Y: MPGN type 3 associated with pemphigus herpetiformis mimicking PGNMID and dermatitis herpetiformis. Case Rep Nephrol Dial. 9:15–24. 2019.PubMed/NCBI View Article : Google Scholar

28 

Mihai S and Sitaru C: Immunopathology and molecular diagnosis of autoimmune bullous diseases. J Cell Mol Med. 11:462–481. 2007.PubMed/NCBI View Article : Google Scholar

29 

Aoki V, Sousa JX Jr, Fukumori LM, Périgo AM, Freitas EL and Oliveira ZN: Direct and indirect immunofluorescence. An Bras Dermatol. 85:490–500. 2010.PubMed/NCBI View Article : Google Scholar : (In English, Portuguese).

30 

Mihályi L, Kiss M, Dobozy A, Kemény L and Husz S: Clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis. Clin Dev Immunol. 2012(369546)2012.PubMed/NCBI View Article : Google Scholar

31 

Saschenbrecker S, Karl I, Komorowski L, Probst C, Dähnrich C, Fechner K, Stöcker W and Schlumberger W: Serological diagnosis of autoimmune bullous skin diseases. Front Immunol. 10(1974)2019.PubMed/NCBI View Article : Google Scholar

32 

Abasq C, Mouquet H, Gilbert D, Tron F, Grassi V, Musette P and Joly P: ELISA testing of anti-desmoglein 1 and 3 antibodies in the management of pemphigus. Arch Dermatol. 145:529–535. 2009.PubMed/NCBI View Article : Google Scholar

33 

Mortazavi H, Shahdi M, Amirzargar AA, Naraghi ZS, Valikhani M, Daneshpazhooh M, Vasheghani-Farahani A, Sedaghat M and Chams-Davatchi C: Desmoglein ELISA in the diagnosis of pemphigus and its correlation with the severity of pemphigus vulgaris. Iran J Allergy Asthma Immunol. 8:53–56. 2009.PubMed/NCBI

34 

Tampoia M, Giavarina D, Di Giorgio C and Bizzaro N: Diagnostic accuracy of enzyme-linked immunosorbent assays (ELISA) to detect anti-skin autoantibodies in autoimmune blistering skin diseases: A systematic review and meta-analysis. Autoimmun Rev. 12:121–126. 2012.PubMed/NCBI View Article : Google Scholar

35 

Naseer SY, Seiffert-Sinha K and Sinha AA: Detailed profiling of anti-desmoglein autoantibodies identifies anti-Dsg1 reactivity as a key driver of disease activity and clinical expression in pemphigus vulgaris. Autoimmunity. 48:231–241. 2015.PubMed/NCBI View Article : Google Scholar

36 

Di Zenzo G, Di Lullo G, Corti D, Calabresi V, Sinistro A, Vanzetta F, Didona B, Cianchini G, Hertl M, Eming R, et al: Pemphigus autoantibodies generated through somatic mutations target the desmoglein-3 cis-interface. J Clin Invest. 122:3781–3790. 2012.PubMed/NCBI View Article : Google Scholar

37 

Lakshmi MJD, Jaisankar TJ, Rajappa M, Thappa DM, Chandrashekar L, Divyapriya D, Munisamy M and Revathy G: Correlation of antimuscarinic acetylcholine receptor antibody titers and antidesmoglein antibody titers with the severity of disease in patients with pemphigus. J Am Acad Dermatol. 76:895–902. 2017.PubMed/NCBI View Article : Google Scholar

38 

Heelan K, Mahar AL, Walsh S and Shear NH: Pemphigus and associated comorbidities: A cross-sectional study. Clin Exp Dermatol. 40:593–599. 2015.PubMed/NCBI View Article : Google Scholar

39 

Parameswaran A, Attwood K, Sato R, Seiffert-Sinha K and Sinha AA: Identification of a new disease cluster of pemphigus vulgaris with autoimmune thyroid disease, rheumatoid arthritis and type I diabetes. Br J Dermatol. 172:729–738. 2015.PubMed/NCBI View Article : Google Scholar

40 

Almugairen N, Hospital V, Bedane C, Duvert-Lehembre S, Picard D, Tronquoy AF, Houivet E, D'incan M and Joly P: Assessment of the rate of long-term complete remission off therapy in patients with pemphigus treated with different regimens including medium- and high-dose corticosteroids. J Am Acad Dermatol. 69:583–588. 2013.PubMed/NCBI View Article : Google Scholar

41 

Satyanarayanasetty D, Pawar K, Nadig P and Haran A: Multiple adverse effects of systemic corticosteroids: A case report. J Clin Diagn Res. 9:FD01–FD2. 2015.PubMed/NCBI View Article : Google Scholar

42 

Ahmed AR, Kaveri S and Spigelman Z: Long-term remissions in recalcitrant pemphigus vulgaris. N Engl J Med. 373:2693–2694. 2015.PubMed/NCBI View Article : Google Scholar

43 

Beissert S, Werfel T, Frieling U, Böhm M, Sticherling M, Stadler R, Zillikens D, Rzany B, Hunzelmann N, Meurer M, et al: A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of pemphigus. Arch Dermatol. 142:1447–1454. 2006.PubMed/NCBI View Article : Google Scholar

44 

Amagai M, Ikeda S, Shimizu H, Iizuka H, Hanada K, Aiba S, Kaneko F, Izaki S, Tamaki K, Ikezawa Z, et al: Pemphigus study group. A randomized double-blind trial of intravenous immunoglobulin for pemphigus. J Am Acad Dermatol. 60:595–603. 2009.PubMed/NCBI View Article : Google Scholar

45 

Higashihara T, Kawase M, Kobayashi M, Hara M, Matsuzaki H, Uni R, Matsumura M, Etoh T and Takano H: Evaluating the efficacy of double-filtration plasmapheresis in treating five patients with drug-resistant pemphigus. Ther Apher Dial. 21:243–247. 2017.PubMed/NCBI View Article : Google Scholar

46 

Eming R and Hertl M: Immunoadsorption in pemphigus. Autoimmunity. 39:609–616. 2006.PubMed/NCBI View Article : Google Scholar

47 

Du FH, Mills EA and Mao-Draayer Y: Next-generation anti-CD20 monoclonal antibodies in autoimmune disease treatment. Auto Immun Highlights. 8(12)2017.PubMed/NCBI View Article : Google Scholar

48 

Wang HH, Liu CW, Li YC and Huang YC: Efficacy of rituximab for pemphigus: A systematic review and meta-analysis of different regimens. Acta Derm Venereol. 95:928–932. 2015.PubMed/NCBI View Article : Google Scholar

49 

Seyfizadeh N, Seyfizadeh N, Hasenkamp J and Huerta-Yepez S: A molecular perspective on rituximab: A monoclonal antibody for B cell non Hodgkin lymphoma and other affections. Crit Rev Oncol Hematol. 97:275–290. 2016.PubMed/NCBI View Article : Google Scholar

50 

Ahmed AR, Spigelman Z, Cavacini LA and Posner MR: Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med. 355:1772–1779. 2006.PubMed/NCBI View Article : Google Scholar

51 

Schmidt E, Goebeler M and Zillikens D: Rituximab in severe pemphigus. Ann N Y Acad Sci. 1173:683–691. 2009.PubMed/NCBI View Article : Google Scholar

52 

Chiu HY, Chang CY, Hsiao CH and Wang LF: Concurrent cytomegalovirus and herpes simplex virus infection in pemphigus vulgaris treated with rituximab and prednisolone. Acta Derm Venereol. 93:200–201. 2013.PubMed/NCBI View Article : Google Scholar

53 

Wei KC, Chen W, Tang PL and Huang YT: Pneumocystis jirovecii pneumonia infection in pemphigus patients treated with rituximab: An observational nationwide epidemiological study in Taiwan. Eur J Dermatol. 28:713–715. 2018.PubMed/NCBI View Article : Google Scholar

54 

Amber KT, Kodiyan J, Bloom R and Hertl M: The controversy of hepatitis C and rituximab: A multidisciplinary dilemma with implications for patients with pemphigus. Indian J Dermatol Venereol Leprol. 82:182–183. 2016.PubMed/NCBI View Article : Google Scholar

55 

Frampton JE: Rituximab: A review in pemphigus vulgaris. Am J Clin Dermatol. 21:149–156. 2020.PubMed/NCBI View Article : Google Scholar

56 

Rapp M, Pentland A and Richardson C: Successful treatment of pemphigus vulgaris with ofatumumab. J Drugs Dermatol. 17:1338–1339. 2018.PubMed/NCBI

57 

Ellebrecht CT, Choi EJ, Allman DM, Tsai DE, Wegener WA, Goldenberg DM and Payne AS: Subcutaneous veltuzumab, a humanized anti-CD20 antibody, in the treatment of refractory pemphigus vulgaris. JAMA Dermatol. 150:1331–1335. 2014.PubMed/NCBI View Article : Google Scholar

58 

Vinay K, Kanwar AJ, Mittal A, Dogra S, Minz RW and Hashimoto T: Intralesional rituximab in the treatment of refractory oral pemphigus vulgaris. JAMA Dermatol. 151:878–882. 2015.PubMed/NCBI View Article : Google Scholar

59 

Paolino G, Didona D, Magliulo G, Iannella G, Didona B, Raffaele S, Moliterni E, Donati M, Ciofalo A, Granata G, et al: Paraneoplastic pemphigus: Insight into the autoimmune pathogenesis, clinical features and therapy. Int J Mol Sci. 18(2532)2017.PubMed/NCBI View Article : Google Scholar

60 

Tsuchisaka A, Numata S, Teye K, Natsuaki Y, Kawakami T, Takeda Y, Wang W, Ishikawa K, Goto M, Koga H, et al: Epiplakin is a paraneoplastic pemphigus autoantigen and related to bronchiolitis obliterans in Japanese patients. J Investig Dermatol. 136:399–408. 2016.PubMed/NCBI View Article : Google Scholar

61 

Czernik A, Camilleri M, Pittelkow MR and Grando SA: Paraneoplastic autoimmune multiorgan syndrome: 20 years after. Int J Dermatol. 50:905–914. 2011.PubMed/NCBI View Article : Google Scholar

62 

Wade MS and Black MM: Paraneoplastic pemphigus: A brief update. Australas J Dermatol. 46:1–8; quiz 9-10. 2005.PubMed/NCBI View Article : Google Scholar

63 

Martel P, Loiseau P, Joly P, Busson M, Lepage V, Mouquet H, Courville P, Flageul B, Charron D, Musette P, et al: Paraneoplastic pemphigus is associated with the DRB1*03 allele. J Autoimmun. 20:91–95. 2003.PubMed/NCBI View Article : Google Scholar

64 

Kim JH and Kim SC: Paraneoplastic pemphigus: Paraneoplastic autoimmune disease of the skin and mucosa. Front Immunol. 10(1259)2019.PubMed/NCBI View Article : Google Scholar

65 

Healy WJ, Peters S and Nana-Sinkam SP: A middle-aged man presenting with unexplained mucosal erosions and progressive dyspnoea. BMJ Case Rep. 2015(bcr2014208677)2015.PubMed/NCBI View Article : Google Scholar

66 

Mar WA, Glaesser R, Struble K, Stephens-Groff S, Bangert J and Hansen RC: Paraneoplastic pemphigus with bronchiolitis obliterans in a child. Pediatr Dermatol. 20:238–242. 2003.PubMed/NCBI View Article : Google Scholar

67 

Wieczorek M and Czernik A: Paraneoplastic pemphigus: A short review. Clin Cosmet Investig Dermatol. 9:291–295. 2016.PubMed/NCBI View Article : Google Scholar

68 

Cummins DL, Mimouni D, Tzu J, Owens N, Anhalt GJ and Meyerle JH: Lichenoid paraneoplastic pemphigus in the absence of detectable antibodies. J Am Acad Dermatol. 56:153–159. 2007.PubMed/NCBI View Article : Google Scholar

69 

Hashimoto T, Amagai M, Watanabe K, Chorzelski TP, Bhogal BS, Black MM, Stevens HP, Boorsma DM, Korman NJ, Gamou S, et al: Characterization of paraneoplastic pemphigus autoantigens by immunoblot analysis. J Invest Dermatol. 104:829–834. 1995.PubMed/NCBI View Article : Google Scholar

70 

Frew JW and Murrell DF: Current management strategies in paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome). Dermatol Clin. 29:607–612. 2011.PubMed/NCBI View Article : Google Scholar

71 

Lee A, Sandhu S, Imlay-Gillespie L, Mulligan S and Shumack S: Successful use of Bruton's kinase inhibitor, ibrutinib, to control paraneoplastic pemphigus in a patient with paraneoplastic autoimmune multiorgan syndrome and chronic lymphocytic leukaemia. Australas J Dermatol. 58:e240–e242. 2017.PubMed/NCBI View Article : Google Scholar

72 

Hohwy T, Bang K, Steiniche T, Peterslund NA and d'Amore F: Alemtuzumab-induced remission of both severe paraneoplastic pemphigus and leukaemic bone marrow infiltration in a case of treatment-resistant B-cell chronic lymphocytic leukaemia. Eur J Haematol. 73:206–209. 2004.PubMed/NCBI View Article : Google Scholar

73 

Gu L and Ye S: Tocilizumab cannot prevent the development of bronchiolitis obliterans in patients with Castleman disease-associated paraneoplastic pemphigus. J Clin Rheumatol. 25:e77–e78. 2019.PubMed/NCBI View Article : Google Scholar

74 

James KA, Culton DA and Diaz LA: Diagnosis and clinical features of pemphigus foliaceus. Dermatol Clin. 29:405–412, viii. 2011.PubMed/NCBI View Article : Google Scholar

75 

Bastuji-Garin S, Souissi R, Blum L, Turki H, Nouira R, Jomaa B, Zahaf A, Ben Osman A, Mokhtar I, Fazaa B, et al: Comparative epidemiology of pemphigus in Tunisia and France: Unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol. 104:302–305. 1995.PubMed/NCBI View Article : Google Scholar

76 

Shirakata Y, Amagai M, Hanakawa Y, Nishikawa T and Hashimoto K: Lack of mucosal involvement in pemphigus foliaceus may be due to low expression of desmoglein 1. J Invest Dermatol. 110:76–78. 1998.PubMed/NCBI View Article : Google Scholar

77 

Abida O, Kallel-Sellami M, Joly P, Ben Ayed M, Zitouni M, Masmoudi A, Mokni M, Fezzaa B, Ben Osman A, Kammoun MR, et al: Franco-Tunisian group of survey and research on pemphigus. Anti-desmoglein 1 antibodies in healthy related and unrelated subjects and patients with pemphigus foliaceus in endemic and non-endemic areas from Tunisia. J Eur Acad Dermatol Venereol. 23:1073–1078. 2009.PubMed/NCBI View Article : Google Scholar

78 

Matkaluk RM and Bailin PL: Penicillamine-induced pemphigus foliaceus. A fatal outcome. Arch Dermatol. 117:156–157. 1981.PubMed/NCBI

79 

Aoki V, Millikan RC, Rivitti EA, Hans-Filho G, Eaton DP, Warren SJ, Li N, Hilario-Vargas J, Hoffmann RG and Diaz LA: Cooperative Group for Fogo Selvagem Research. Environmental risk factors in endemic pemphigus foliaceus (fogo selvagem). J Investig Dermatol Symp Proc. 9:34–40. 2004.PubMed/NCBI View Article : Google Scholar

80 

Dalla-Costa R, Pincerati MR, Beltrame MH, Malheiros D and Petzl-Erler ML: Polymorphisms in the 2q33 and 3q21 chromosome regions including T-cell coreceptor and ligand genes may influence susceptibility to pemphigus foliaceus. Hum Immunol. 71:809–817. 2010.PubMed/NCBI View Article : Google Scholar

81 

Bruckner N, Katz RA and Hood AF: Pemphigus foliaceus resembling eruptive seborrheic keratoses. Arch Dermatol. 116:815–816. 1980.PubMed/NCBI

82 

Jacyk WK and Simson IW: Pemphigus erythematosus resembling multiple seborrheic keratoses. Arch Dermatol. 126:543–544. 1990.PubMed/NCBI View Article : Google Scholar

83 

Metry DW, Hebert AA and Jordon RE: Nonendemic pemphigus foliaceus in children. J Am Acad Dermatol. 46:419–422. 2002.PubMed/NCBI View Article : Google Scholar

84 

Gomi H, Kawada A, Amagai M and Matsuo I: Pemphigus erythematosus: Detection of anti-desmoglein-1 antibodies by ELISA. Dermatology. 199:188–189. 1999.PubMed/NCBI View Article : Google Scholar

85 

Qaqish BF, Prisayanh P, Qian Y, Andraca E, Li N, Aoki V, Hans-Filho G, dos Santos V, Rivitti EA and Diaz LA: Cooperative Group on Fogo Selvagem Research. Development of an IgG4-based predictor of endemic pemphigus foliaceus (fogo selvagem). J Invest Dermatol. 129:110–118. 2009.PubMed/NCBI View Article : Google Scholar

86 

Schmidt E, Dähnrich C, Rosemann A, Probst C, Komorowski L, Saschenbrecker S, Schlumberger W, Stöcker W, Hashimoto T, Bröcker EB, et al: Novel ELISA systems for antibodies to desmoglein 1 and 3: Correlation of disease activity with serum autoantibody levels in individual pemphigus patients. Exp Dermatol. 19:458–463. 2010.PubMed/NCBI View Article : Google Scholar

87 

Sar-Pomian M, Kurzeja M, Rudnicka L and Olszewska M: The value of trichoscopy in the differential diagnosis of scalp lesions in pemphigus vulgaris and pemphigus foliaceus. An Bras Dermatol. 89:1007–1012. 2014.PubMed/NCBI View Article : Google Scholar

88 

Sami N, Qureshi A and Ahmed AR: Steroid sparing effect of intravenous immunoglobulin therapy in patients with pemphigus foliaceus. Eur J Dermatol. 12:174–178. 2002.PubMed/NCBI

89 

Sawamura S, Kajihara I, Makino K, Makino T, Fukushima S, Jinnin M, Oyama B, Hashimoto T and Ihn H: Systemic lupus erythematosus associated with myasthenia gravis, pemphigus foliaceus and chronic thyroiditis after thymectomy. Australas J Dermatol. 58:e120–e122. 2017.PubMed/NCBI View Article : Google Scholar

90 

Kridin K, Patel PM, Jones VA, Cordova A and Amber KT: IgA pemphigus: A systematic review. J Am Acad Dermatol. 82:1386–1392. 2020.PubMed/NCBI View Article : Google Scholar

91 

Düker I, Schaller J, Rose C, Zillikens D, Hashimoto T and Kunze J: Subcorneal pustular dermatosis–type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3. Arch Dermatol. 145:1159–1162. 2009.PubMed/NCBI View Article : Google Scholar

92 

Watts PJ and Khachemoune A: Subcorneal pustular dermatosis: A review of 30 years of progress. Am J Clin Dermatol. 17:653–671. 2016.PubMed/NCBI View Article : Google Scholar

93 

Kuan YZ, Chiou HT, Chang HC, Chan HL and Kuo TT: Intraepidermal neutrophilic IgA dermatosis. J Am Acad Dermatol. 22:917–919. 1990.PubMed/NCBI View Article : Google Scholar

94 

Adam Z, Krejcí M, Pour L, Feit J, Büchler T and Hájek R: IgA pemphigus associated with monoclonal gammopathy completely resolved after achievement of complete remission of multiple myeloma with bortezomib, cyclophosphamide and dexamethasone regimen. Wien Klin Wochenschr. 122:311–314. 2010.PubMed/NCBI View Article : Google Scholar

95 

Hirata Y, Abe R, Kikuchi K, Hamasaka A, Shinkuma S, Ujiie H, Nomura T, Nishie W, Arita K and Shimizu H: Intraepidermal neutrophilic IgA pemphigus successfully treated with dapsone. Eur J Dermatol. 22:282–283. 2012.PubMed/NCBI View Article : Google Scholar

96 

Howell SM, Bessinger GT, Altman CE and Belnap CM: Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil. J Am Acad Dermatol. 53:541–543. 2005.PubMed/NCBI View Article : Google Scholar

97 

Bliziotis I, Rafailidis P, Vergidis P and Falagas ME: Regression of subcorneal pustular dermatosis type of IgA pemphigus lesions with azithromycin. J Infect. 51:E31–E34. 2005.PubMed/NCBI View Article : Google Scholar

98 

Gruss C, Zillikens D, Hashimoto T, Amagai M, Kroiss M, Vogt T, Landthaler M and Stolz W: Rapid response of IgA pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin. J Am Acad Dermatol. 43:923–926. 2000.PubMed/NCBI View Article : Google Scholar

Related Articles

  • Abstract
  • View
  • Download
  • Twitter
Copy and paste a formatted citation
Spandidos Publications style
Costan V, Popa C, Hâncu MF, Porumb‑Andrese E and Toader MP: Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review). Exp Ther Med 22: 1335, 2021.
APA
Costan, V., Popa, C., Hâncu, M.F., Porumb‑Andrese, E., & Toader, M.P. (2021). Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review). Experimental and Therapeutic Medicine, 22, 1335. https://doi.org/10.3892/etm.2021.10770
MLA
Costan, V., Popa, C., Hâncu, M. F., Porumb‑Andrese, E., Toader, M. P."Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review)". Experimental and Therapeutic Medicine 22.5 (2021): 1335.
Chicago
Costan, V., Popa, C., Hâncu, M. F., Porumb‑Andrese, E., Toader, M. P."Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review)". Experimental and Therapeutic Medicine 22, no. 5 (2021): 1335. https://doi.org/10.3892/etm.2021.10770
Copy and paste a formatted citation
x
Spandidos Publications style
Costan V, Popa C, Hâncu MF, Porumb‑Andrese E and Toader MP: Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review). Exp Ther Med 22: 1335, 2021.
APA
Costan, V., Popa, C., Hâncu, M.F., Porumb‑Andrese, E., & Toader, M.P. (2021). Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review). Experimental and Therapeutic Medicine, 22, 1335. https://doi.org/10.3892/etm.2021.10770
MLA
Costan, V., Popa, C., Hâncu, M. F., Porumb‑Andrese, E., Toader, M. P."Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review)". Experimental and Therapeutic Medicine 22.5 (2021): 1335.
Chicago
Costan, V., Popa, C., Hâncu, M. F., Porumb‑Andrese, E., Toader, M. P."Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review)". Experimental and Therapeutic Medicine 22, no. 5 (2021): 1335. https://doi.org/10.3892/etm.2021.10770
Follow us
  • Twitter
  • LinkedIn
  • Facebook
About
  • Spandidos Publications
  • Careers
  • Cookie Policy
  • Privacy Policy
How can we help?
  • Help
  • Live Chat
  • Contact
  • Email to our Support Team