Skip to main content

Advertisement

Log in

Esophagogastroduodenoscopy and Esophageal Involvement in Patients with Pemphigus Vulgaris

  • Original Article
  • Published:
Dysphagia Aims and scope Submit manuscript

Abstract

Pemphigus vulgaris (PV) is a rare autoimmune blistering disease involving the skin and mucous membranes. The prevalence of esophageal involvement remains uncertain. The aim of our study was to determine the frequency of esophageal involvement in patients with PV. This is a single-center electronic database retrospective review of patients with a diagnosis of PV. Data abstracted included demographics, disease characteristics (biopsy results, symptoms, areas affected, treatments), and esophagogastroduodenoscopy (EGD) reports. Of the 111 patients that met eligibility criteria, only 22 (19.8%) underwent EGD. Demographic data were similar except those who underwent EGD were more likely to be female (77.3% vs. 51.7%, p = 0.05) and have hypertension (50.0% vs. 24.7%, p = 0.04). Esophageal symptoms were common in both groups; however, those experiencing dysphagia were more likely to undergo EGD (50.0% vs. 20.2%, p = 0.007). Those who underwent EGD had more refractory disease (≥ 3 treatment modalities: 100% vs. 58.4%, p < 0.001), but did not differ in areas affected. Of those who underwent EGD, only 4 (18.2%) had esophageal abnormalities either prior to PV diagnosis (1) or during a disease flare (3). Those having a flare were more likely to experience odynophagia (69.2%) or weight loss (61.5%), p = 0.02 and p = 0.05, respectively. While esophageal symptoms were common in our cohort of PV patients, a minority of patients underwent EGD, and the vast majority of those were unremarkable. This suggests that while esophageal symptoms are common in PV, permanent esophageal injury is more rare.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Mihai S, Sitaru C. Immunopathology and molecular diagnosis of autoimmune bullous diseases. J Cell Mol Med. 2007;11(3):462–81. https://doi.org/10.1111/j.1582-4934.2007.00033.x.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Nishikawa T, Hashimoto T, Shimizu H, Ebihara T, Amagai M. Pemphigus: from immunofluorescence to molecular biology. J Dermatol Sci. 1996;12(1):1–9.

    Article  CAS  Google Scholar 

  3. Bystryn JC, Rudolph JL. Pemphigus. Lancet. 2005;366(9479):61–73. https://doi.org/10.1016/S0140-6736(05)66829-8.

    Article  PubMed  Google Scholar 

  4. Mustafa MB, Porter SR, Smoller BR, Sitaru C. Oral mucosal manifestations of autoimmune skin diseases. Autoimmun Rev. 2015;14(10):930–51. https://doi.org/10.1016/j.autrev.2015.06.005.

    Article  PubMed  Google Scholar 

  5. Kavala M, Altintas S, Kocaturk E, Zindanci I, Can B, Ruhi C, Turkoglu Z. Ear, nose and throat involvement in patients with pemphigus vulgaris: correlation with severity, phenotype and disease activity. J Eur Acad Dermatol Venereol. 2011;25(11):1324–7. https://doi.org/10.1111/j.1468-3083.2011.03981.x.

    Article  CAS  PubMed  Google Scholar 

  6. Kavala M, Topaloglu Demir F, Zindanci I, Can B, Turkoglu Z, Zemheri E, Cam OH, Teksen A. Genital involvement in pemphigus vulgaris (PV): correlation with clinical and cervicovaginal Pap smear findings. J Am Acad Dermatol. 2015;73(4):655–9. https://doi.org/10.1016/j.jaad.2015.06.057.

    Article  PubMed  Google Scholar 

  7. Bystryn JC, Steinman NM. The adjuvant therapy of pemphigus. An update. Arch Dermatol. 1996;132(2):203–12.

    Article  CAS  Google Scholar 

  8. Risser J, Lewis K, Weinstock MA. Mortality of bullous skin disorders from 1979 through 2002 in the United States. Arch Dermatol. 2009;145(9):1005–8. https://doi.org/10.1001/archdermatol.2009.205.

    Article  PubMed  Google Scholar 

  9. Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJ, West J. Bullous pemphigoid and pemphigus vulgaris–incidence and mortality in the UK: population based cohort study. BMJ. 2008;337:a180. https://doi.org/10.1136/bmj.a180.

    Article  CAS  PubMed  Google Scholar 

  10. Galloro G, Mignogna M, de Werra C, Magno L, Diamantis G, Ruoppo E, Iovino P. The role of upper endoscopy in identifying oesophageal involvement in patients with oral pemphigus vulgaris. Dig Liver Dis. 2005;37(3):195–9. https://doi.org/10.1016/j.dld.2004.10.005.

    Article  CAS  PubMed  Google Scholar 

  11. Calka O, Akdeniz N, Tuncer I, Metin A, Cesur RS. Oesophageal involvement during attacks in pemphigus vulgaris patients. Clin Exp Dermatol. 2006;31(4):515–9. https://doi.org/10.1111/j.1365-2230.2006.02132.x.

    Article  CAS  PubMed  Google Scholar 

  12. Okamura A, Nakamura R, Yamagami J, Ishii K, Kawakubo H, Omori T, Takeuchi H, Amagai M, Kitagawa Y. Evaluation of pharyngo-oesophageal involvement in pemphigus vulgaris and its correlation with disease activity. Br J Dermatol. 2017;176(1):224–6. https://doi.org/10.1111/bjd.14725.

    Article  CAS  PubMed  Google Scholar 

  13. Rao PN, Samarth A, Aurangabadkar SJ, Pratap B, Lakshmi TS. Study of upper gastrointestinal tract involvement in pemphigus by esophago-gastro-duodenoscopy. Indian J Dermatol Venereol Leprol. 2006;72(6):421–4.

    Article  Google Scholar 

  14. Mignogna MD, Lo Muzio L, Galloro G, Satriano RA, Ruocco V, Bucci E. Oral pemphigus: clinical significance of esophageal involvement: report of eight cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1997;84(2):179–84.

    Article  CAS  Google Scholar 

  15. Lundell LR, Dent J, Bennett JR, Blum AL, Armstrong D, Galmiche JP, Johnson F, Hongo M, Richter JE, Spechler SJ, Tytgat GN, Wallin L. Endoscopic assessment of oesophagitis: clinical and functional correlates and further validation of the Los Angeles classification. Gut. 1999;45(2):172–80.

    Article  CAS  Google Scholar 

  16. Raque CJ, Stein KM, Samitz MH. Pemphigus vulagis involving the esophagus. Arch Dermatol. 1970;102(4):371–3.

    Article  CAS  Google Scholar 

  17. Kaplan RP, Touloukian J, Ahmed AR, Newcomer VD. Esophagitis dissecans superficialis associated with pemphigus vulgaris. J Am Acad Dermatol. 1981;4(6):682–7.

    Article  CAS  Google Scholar 

  18. Palleschi GM, Cipollini EM, Lotti T. Development of oesophageal involvement in a subject with pemphigus vulgaris: a case report and review of the literature. J Eur Acad Dermatol Venereol. 2002;16(4):405–8.

    Article  CAS  Google Scholar 

  19. Trattner A, Lurie R, Leiser A, David M, Hazaz B, Kadish U, Sandbank M. Esophageal involvement in pemphigus vulgaris: a clinical, histologic, and immunopathologic study. J Am Acad Dermatol. 1991;24(2 Pt 1):223–6.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Katharine A. Ozeki.

Ethics declarations

Conflict of interest

The authors have no conflict of interest to disclose.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Ozeki, K.A., Zikos, T.A., Clarke, J.O. et al. Esophagogastroduodenoscopy and Esophageal Involvement in Patients with Pemphigus Vulgaris. Dysphagia 35, 503–508 (2020). https://doi.org/10.1007/s00455-019-10055-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00455-019-10055-4

Keywords

Navigation