Demographic, Clinical, and Paraclinical Characteristics of Patients with Pemphigus Vulgaris Needing Hospital Admission during the Years 2006-2017

Document Type : Original Article (s)

Authors

1 Associate Professor, Department of Dermatology, School of Medicine, Alzahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran

2 Professor, Department of Dermatology, School of Medicine, Alzahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran

3 Student of Medicine, Student Research Committee, Isfahan University of Medical Sciences, Isfahan, Iran

Abstract

Background: Pemphigus vulgaris is an autoimmune disorder, which appears as a mucosal intracutaneous blister. The disease prevalence is different in various countries and regions. Therefore, identification of the prevalence and characteristics of patients with this disease in different climates can be useful in initiating early treatment and management, and reducing the mortality. So, the aim of this study was to evaluate the epidemiology of Pemphigus vulgaris in Isfahan City, Iran during a ten-year period.Methods: In this descriptive retrospective study, 114 patients with Pemphigus vulgaris who were admitted in Alzahra hospital in Isfahan during the years of 2006-2017 were studied. Demographic, clinical, and paraclinical data were recorded, and the results were reported using SPSS software.Findings: Of 114 patients, 78 (68.4%) were women and (31.6%) 36 were men, with a mean age of 49.15 ± 12.55 years. Type of the lesion was mucosal and vegetative in 14.9%, cutaneous in 24.4%, and both mucosal and cutaneous in 60.5%. In addition, the prevalence of this disease in the spring and summer was 31.6% and 27.2%, respectively. Most patients were treated with prednisolone and Azaram drugs, which were associated with complications in 17.8%; but eventually all patients recovered and no mortality occurred during this assessment.Conclusion: According to the results of the study, the prevalence of this disease in women was more than men, and most of them appeared in spring and summer as cutaneous-mucosal manifestations. Therefore, it seems that more research is needed on the prevalence of this disease and its manifestations, the season of more prevalence, and successful treatments.

Keywords


  1. Tavakolpour S. Pemphigus trigger factors: Special focus on pemphigus vulgaris and pemphigus foliaceus. Arch Dermatol Res 2018; 310(2): 95-106.
  2. Miyamoto S, Ohkubo A, Seshima H, Komori S, Yamamoto M, Maeda T, et al. Selective Plasma Exchange for the Removal of Pemphigus Autoantibodies, Fibrinogen, and Factor XIII in Pemphigus Vulgaris. Ther Apher Dial 2017; 21(3): 226-31.
  3. Tsankov N, Vassileva S, Kamarashev J, Kazandjieva J, Kuzeva V. Epidemiology of pemphigus in Sofia, Bulgaria. A 16-year retrospective study (1980-1995). Int J Dermatol 2000; 39(2): 104-8.
  4. Salmanpour R, Shahkar H, Namazi MR, Rahman-Shenas MR. Epidemiology of pemphigus in south-western Iran: a 10-year retrospective study (1991-2000). Int J Dermatol 2006; 45(2): 103-5.
  5. Chams-Davatchi C, Valikhani M, Daneshpazhooh M, Esmaili N, Balighi K, Hallaji Z, et al. Pemphigus: analysis of 1209 cases. Int J Dermatol 2005; 44(6): 470-6.
  6. Kridin K, Zelber-Sagi S, Bergman R. Pemphigus
  7. vulgaris and pemphigus foliaceus: Differences in epidemiology and mortality. Acta Derm Venereol 2017; 97(9): 1095-9.
  8. Korman NJ. New immunomodulating drugs in autoimmune blistering diseases. Dermatol Clin 2001; 19(4): 637-48.
  9. Halaji Z, Esmaili N, Moeineddin F, Zeinali F, Aalami L, Shams-Davatchi S. Prognostic factors of pemphigus vulgaris disease: A study on 119 patients. Tehran Univ Med J 2009; 66(12): 913-8. [In Persian].
  10. Bastuji-Garin S, Turki H, Mokhtar I, Nouira R, Fazaa B, Jomaa B, et al. Possible relation of Tunisian pemphigus with traditional cosmetics: a multicenter case-control study. Am J Epidemiol 2002; 155(3): 249-56.
  11. Jang HS, Oh CK, Lim JY, Jun ES, Kim YS, Kwon KS. Detection of human herpesvirus 8 DNA in pemphigus and chronic blistering skin diseases. J Korean Med Sci 2000; 15(4): 442-8.
  12. Goon AT, Tan SH. Comparative study of pemphigus vulgaris and pemphigus foliaceus in Singapore. Australas J Dermatol 2001; 42(3): 172-5.
  13. Seo PG, Choi WW, Chung JH. Pemphigus in Korea: clinical manifestations and treatment protocol. J Dermatol 2003; 30(11): 782-8.
  14. Micali G, Musumeci ML, Nasca MR. Epidemiologic analysis and clinical course of 84 consecutive cases of pemphigus in eastern Sicily. Int J Dermatol 1998; 37(3): 197-200.
  15. Ellebrecht CT, Payne AS. Setting the target for pemphigus vulgaris therapy. JCI Insight 2017; 2(5): e92021.
  16. Svecova D. Pemphigus vulgaris: A clinical study of 44 cases over a 20-year period. Int J Dermatol 2015; 54(10): 1138-44.
  17. Saleh MA. Pemphigus in the Arab world. J Dermatol 2015; 42(1): 27-30.
  18. Asilian A, Yoosefi A, Faghini G. Pemphigus vulgaris in Iran: epidemiology and clinical profile. Skinmed 2006; 5(2): 69-71.
  19. Sadr Eshkevari S, Maboudi A, Akbari Dastak E, Golchai J, Shams Guilani J, Dorjani A, et al. Pemphigus in Guilan: Clinical and epidemiologic features of 126 hospitalized patients. Iran J Dermato 2005; 8(30): 104-9.
  20. Chams-Davatchi C, Nonahal Azar R, Daneshpazooh M, Valikhani M, Hallaji Z, Barzegari M, et al. Open trial of mycophenolate mofetil in the treatment of resistant pemphigus vulgaris. Ann Dermatol Venereol
  21. ; 129(1 Pt 1): 23-5.
  22. Baum S, Astman N, Berco E, Solomon M, Trau H, Barzilai A. Epidemiological data of 290 pemphigus vulgaris patients: A 29-year retrospective study. Eur J Dermatol 2016; 26(4): 382-7.
  23. Shah AA, Seiffert-Sinha K, Sirois D, Werth VP, Rengarajan B, Zrnchik W, et al. Development of a disease registry for autoimmune bullous diseases: initial analysis of the pemphigus vulgaris subset. Acta Derm Venereol 2015; 95(1): 86-90.
  24. Yaghoubi R. Pemphigus disease in Khuzestan: Study of 111 cases. Iran J Dermato 1999; 3(1): 16-23. [In Persian].
  25. Krain LS. Pemphigus. Epidemiologic and survival characteristics of 59 patients, 1955-1973. Arch Dermatol 1974; 110(6): 862-5.
  26. Huang A, Madan RK, Levitt J. Future therapies for pemphigus vulgaris: Rituximab and beyond. J Am Acad Dermatol 2016; 74(4): 746-53.
  27. Tavakolpour S, Mahmoudi H, Balighi K, Abedini R, Daneshpazhooh M. Sixteen-year history of rituximab therapy for 1085 pemphigus vulgaris patients: A systematic review. Int Immunopharmacol 2018; 54: 131-8.
  28. Seidenbaum M, David M, Sandbank M. The course and prognosis of pemphigus. A review of 115 patients. Int J Dermatol 1988; 27(8): 580-4.