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Differences among Indian and European pemphigus patients based on demographics, clinical parameters and propensity for relapse: results of a prospective bicontinental cohort study Volume 33, issue 6, November-December 2023

Authors
1 Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Dermatology and Venereology, Faculty of Medicine, Medical University – Sofia, Bulgaria
3 Department of Dermatology, University of Lübeck, Lübeck, Germany
4 Department of Endocrinology (Immunology Division), Postgraduate Institute of Medical Education and Research, Chandigarh, India
5 Department of Immunology-Histocompatibility, Papageorgiou General Hospital, Thessaloniki, Greece
6 Department of Clinical Immunology, Faculty of Medicine, Medical University – Sofia, Sofia, Bulgaria
7 2nd University Dermatology Department, Aristotle University School of Medicine, Thessaloniki, Greece
8 Department Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
9 Department of Biostatistics, Postgraduate Institute of Medical Education and Research, Chandigarh, India
10 Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany
* Reprints: Dipankar De
* These authors contributed equally

Background

Limited studies have explored pemphigus variations among different ethnic groups residing in their respective geographical locations.

Objectives

This bicontinental study aimed to compare clinical and immunological parameters in Indian and European pemphigus patients in complete remission, off therapy, or on minimal therapy.

Materials & Methods

105 patients (India, n= 75; Bulgaria, n=15; Greece, n=15) with pemphigus vulgaris (PV) or pemphigus foliaceous (PF) in complete remission on minimal therapy (n=64) or complete remission off therapy (n=41) were recruited. Demographic, clinical, and immunological parameters were compared.

Results

Indian patients were significantly younger, the maximal disease severity during the preceding active disease phase was significantly lower, and treatment duration until complete remission was significantly shorter, compared to European patients. European patients had significantly higher anti-Dsg3 serum levels and higher IgG positivity rate based on direct immunofluorescence microscopy at baseline. Furthermore, European patients revealed higher CD19, CD19+ CD27+ cell counts, compared with patients from India. Of note, none of the European patients (n=30) relapsed within the study period, in contrast to 29/75 (38.6%) Indian patients. Treatment strategies differed significantly between the two cohorts, with more frequent utilization of rituximab to achieve remission in the Indian cohort, while prednisolone was more widely used for maintaining remission in the European cohort.

Conclusion

The observed heterogeneity of pemphigus among patients of different ethnicities in terms of demographics, clinical parameters, and propensity for relapse may be due to genetic background or different treatment strategies.

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