Pemphigus in North India

Abstract

Background: Pemphigus is an autoimmune mucocutaneous blistering disease caused by antibodies against desmogleins Dsg-1 and Dsg-3. The epidemiology of the disease varies in different countries. In India, pemphigus is relatively common, with considerable interstate variation. Objective: The objective of this study was to review the demography, clinical features, and treatment aspects of pemphigus in North India. Methods: The case records of pemphigus patients registered from 1988 to 2004 were retrospectively analyzed. The age, sex, residential particulars, site of onset of disease, duration between involvement of skin and mucosa, subtype and course of the disease, and treatments offered were analyzed. Results: Of the 328 patients, 302 (92%) were pemphigus vulgaris and the remaining 26 (8%) were pemphigus foliaceous patients. The mean age at onset was 39.27 years for males and 38.57 years for females. The majority of patients were from the states of Punjab and Haryana. The majority of patients were treated with dexamethasone-cyclophosphamide pulse (DCP) therapy, and the number of DCPs required for inducing remission correlated roughly with the severity of the disease. The mortality rate was 4% in the total sample. Conclusion: North Indian patients of pemphigus have a relatively younger age at onset. The majority of patients were from the states of Punjab and Haryana. The response to DCP therapy was good, and with DCP, the additional dose of oral steroids that was required to control the activity of the disease was less than 30 mg/d, which is much less than the dosage used in the standard conventional regimens.