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Pemphigus in North India.

Amrinder J Kanwar1, Amrinder C Ajith, Tarun Narang

  • 1Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. kanwaraj@hotmail.com

Journal of Cutaneous Medicine and Surgery
|January 24, 2007
PubMed
Summary
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Pemphigus patients in North India present at a younger age and respond well to dexamethasone-cyclophosphamide pulse (DCP) therapy. This treatment requires lower additional oral steroid doses for remission, indicating its efficacy in managing this autoimmune blistering disease.

Area of Science:

  • Dermatology
  • Immunology
  • Epidemiology

Background:

  • Pemphigus is an autoimmune blistering disease targeting desmogleins Dsg-1 and Dsg-3.
  • Epidemiology of pemphigus shows significant geographic variation, being relatively common in India.

Purpose of the Study:

  • To investigate the demographic characteristics, clinical presentations, and treatment outcomes of pemphigus in North India.

Main Methods:

  • Retrospective analysis of 328 pemphigus patient records from 1988 to 2004.
  • Evaluation of patient demographics, disease onset, subtype, course, and treatment modalities.

Main Results:

  • Pemphigus vulgaris constituted 92% of cases; mean age at onset was ~39 years.
  • Patients predominantly from Punjab and Haryana received dexamethasone-cyclophosphamide pulse (DCP) therapy.

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  • DCP therapy showed good response, with remission correlating to disease severity; mortality rate was 4%.

    • Conclusions:

    • North Indian pemphigus patients exhibit a younger age at onset.
    • Dexamethasone-cyclophosphamide pulse (DCP) therapy is effective, requiring less additional oral steroids compared to conventional regimens.
    • Geographic clustering observed in Punjab and Haryana warrants further epidemiological investigation.